378 results match your criteria Striatonigral Degeneration


Eye movement deficits in X-linked dystonia-parkinsonism are related to striatal degeneration.

Parkinsonism Relat Disord 2018 Oct 15. Epub 2018 Oct 15.

Department of Neurology, University of Luebeck, Luebeck, Germany. Electronic address:

Background: X-linked dystonia-parkinsonism (XDP) is characterized by the unique transition of dystonia to parkinsonism and striatal degeneration. Slowing of saccades on clinical examination has been taken as suggestive of a progressive supranuclear palsy (PSP) phenotype.

Objectives: To elucidate whether eye movement abnormalities in XDP patients reflect striatonigral impairment or deficits in the brainstem saccade generator as present in PSP. Read More

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http://dx.doi.org/10.1016/j.parkreldis.2018.10.016DOI Listing
October 2018
1 Read

Young-onset multiple system atrophy: Clinical and pathological features.

Mov Disord 2018 Jul;33(7):1099-1107

University College London (UCL) Institute of Neurology, London, UK.

Background: The onset of multiple system atrophy (MSA) before age 40 years is referred to as "young-onset MSA." We identified clinical and pathological characteristics that might help with its early diagnosis and distinction from young-onset Parkinson's disease and late-onset MSA.

Methods: We reviewed the available clinical and pathological features in cases that fulfilled consensus criteria for diagnosis of probable MSA or had autopsy confirmed MSA with an onset before age 40 years and compared the clinical features with 16 autopsy confirmed cases with young-onset Parkinson's disease and a large published series of late-onset MSA from the European MSA Study Group. Read More

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http://doi.wiley.com/10.1002/mds.27450
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http://dx.doi.org/10.1002/mds.27450DOI Listing
July 2018
6 Reads

Immunohistochemical and Molecular Investigations Show Alteration in the Inflammatory Profile of Multiple System Atrophy Brain.

J Neuropathol Exp Neurol 2018 Jul;77(7):598-607

Queen Square Brain Bank for Neurological Disorders, Department of Molecular Neuroscience, UCL Institute of Neurology, London, UK.

Multiple system atrophy (MSA) is an adult-onset neurodegenerative disease characterized by aggregation of α-synuclein in oligodendrocytes to form glial cytoplasmic inclusions. According to the distribution of neurodegeneration, MSA is subtyped as striatonigral degeneration (SND), olivopontocerebellar atrophy (OPCA), or as combination of these 2 (mixed MSA). In the current study, we aimed to investigate regional microglial populations and gene expression in the 3 different MSA subtypes. Read More

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http://dx.doi.org/10.1093/jnen/nly035DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6005028PMC
July 2018
14 Reads

Spatial correlation and segregation of multimodal MRI abnormalities in multiple system atrophy.

J Neurol 2018 Jul 25;265(7):1540-1547. Epub 2018 Apr 25.

Department of Neurology, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Beomo-ri, Mulgum-eup, Yangsan, Gyeongsangnam-do, 626-770, Republic of Korea.

Objective: The variability of the severity and regional distribution of pathological process in basal ganglia (BG) and brainstem-cerebellar systems results in clinical heterogeneity and represents the motor subtype of multiple system atrophy (MSA). This study aimed to quantify spatial patterns of multimodal MRI abnormalities in BG and stem-CB regions and define structural MRI findings that correlate with clinical characteristics.

Methods: We simultaneously measured R2*, mean diffusivity (MD), and volume in the subcortical structures (BG, thalamus, brainstem-cerebellar regions) of 39 probable MSA and 22 control subjects. Read More

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http://link.springer.com/10.1007/s00415-018-8874-z
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http://dx.doi.org/10.1007/s00415-018-8874-zDOI Listing
July 2018
5 Reads
3.380 Impact Factor

Patterns of dopamine transporter imaging in subtypes of multiple system atrophy.

Acta Neurol Scand 2018 Aug 24;138(2):170-176. Epub 2018 Mar 24.

Department of Neurology & National Clinical Research Center for Aging and Medicine, Huashan Hospital, Fudan University, Shanghai, China.

Objectives: To investigate the differences in the pattern of striatal (caudate and putamen) dopamine transporter (DAT) loss in a multiple system atrophy (MSA) cohort, based on the clinical variants parkinsonian subtype (MSA-P) and cerebellar subtype (MSA-C) via (11)C-N-2-carbomethoxy-3-(4-fluorophenyl)-tropane ( C-CFT) positron emission tomography (PET) imaging.

Materials And Methods: One hundred and six subjects (forty-one patients with probable MSA-P; forty patients with probable MSA-C; twenty-five healthy controls) underwent C-CFT PET. Subregional C-CFT uptake of bilateral caudate, anterior putamen, and posterior putamen was calculated respectively to measure the striatal dopaminergic function. Read More

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http://dx.doi.org/10.1111/ane.12932DOI Listing
August 2018
2 Reads

LRRK2 p.Ile1371Val Mutation in a Case with Neuropathologically Confirmed Multi-System Atrophy.

J Parkinsons Dis 2018 ;8(1):93-100

Parkinson's Institute and Clinical Center, Sunnyvale, CA, USA.

Background: Mutations in the leucine rich repeat kinase 2 (LRRK2) gene are among the most common genetic causes of Lewy body Parkinson's disease (PD). However, LRRK2 mutations can also lead to a variety of pathological phenotypes other than typical PD, including relatively pure nigrostriatal cell loss without alpha-synuclein-positive Lewy bodies or Lewy neurites, progressive supranuclear palsy (PSP), and multiple system atrophy (MSA). The mechanisms behind this remarkable pleomorphic pathology are currently unclear. Read More

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http://dx.doi.org/10.3233/JPD-171237DOI Listing
January 2018
2 Reads

The Relevance of Iron in the Pathogenesis of Multiple System Atrophy: A Viewpoint.

J Alzheimers Dis 2018 ;61(4):1253-1273

Department of Neurology, Medical University of Innsbruck, Innsbruck, Austria.

Iron is essential for cellular development and maintenance of multiple physiological processes in the central nervous system. The disturbance of its homeostasis leads to abnormal iron deposition in the brain and causes neurotoxicity via generation of free radicals and oxidative stress. Iron toxicity has been established in the pathogenesis of Parkinson's disease; however, its contribution to multiple system atrophy (MSA) remains elusive. Read More

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http://dx.doi.org/10.3233/JAD-170601DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5798525PMC
January 2018
1 Read

Progressive striatonigral degeneration in a transgenic mouse model of multiple system atrophy: translational implications for interventional therapies.

Acta Neuropathol Commun 2018 01 3;6(1). Epub 2018 Jan 3.

Division of Neurobiology, Department of Neurology, Medical University of Innsbruck, Innrain 66 / G2, 6020, Innsbruck, Austria.

Multiple system atrophy (MSA) is a rapidly progressive neurodegenerative disorder characterized by widespread oligodendroglial cytoplasmic inclusions of filamentous α-synuclein, and neuronal loss in autonomic centres, basal ganglia and cerebellar circuits. It has been suggested that primary oligodendroglial α-synucleinopathy may represent a trigger in the pathogenesis of MSA, but the mechanisms underlying selective vulnerability and disease progression are unclear. The post-mortem analysis of MSA brains provides a static final picture of the disease neuropathology, but gives no clear indication on the sequence of pathogenic events in MSA. Read More

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http://dx.doi.org/10.1186/s40478-017-0504-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5753576PMC
January 2018
8 Reads

Neuropathology of childhood-onset basal ganglia degeneration caused by mutation of .

Ann Clin Transl Neurol 2017 12 7;4(12):859-864. Epub 2017 Nov 7.

Bruce Lefroy Centre for Genetic Health Research Murdoch Childrens Research Institute Parkville Victoria 3052 Australia.

Objective: To characterize the clinical features and neuropathology associated with recessive mutations.

Methods: Whole-exome sequencing was used to identify the genetic etiology of a rapidly progressive neurological disease presenting in early childhood in two deceased siblings with distinct neuropathological features on post mortem examination.

Results: We identified compound heterozygous variants in in two deceased siblings with early childhood onset of severe, progressive dystonia, and neurodegeneration. Read More

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http://dx.doi.org/10.1002/acn3.487DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5740235PMC
December 2017
2 Reads

Multiple system atrophy: Building a global community - 30years of advocacy efforts.

Authors:
Pamela G Bower

Auton Neurosci 2018 May 16;211:39-42. Epub 2017 Dec 16.

The Multiple System Atrophy Coalition, Inc., 9935-D Rea Rd, #212, Charlotte, NC 28277, USA. Electronic address:

Multiple system atrophy (MSA) is a rare, progressive and ultimately fatal neurodegenerative disease with no known cause and no available disease modifying treatment. Known previously by various names including Shy-Drager Syndrome, olivopontocerebellar atrophy (OPCA) and striatonigral degeneration, MSA can be classified simultaneously as a movement disorder, an autonomic disorder, a cerebellar ataxia and an atypical parkinsonian disorder. Despite scholarly attempts to better describe the disease, awareness among medical practitioners about multiple system atrophy as a diagnostic possibility has been slow to catch on. Read More

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http://dx.doi.org/10.1016/j.autneu.2017.12.006DOI Listing
May 2018
1 Read

Striatal Gα/cAMP Signal-Dependent Mechanism to Generate Levodopa-Induced Dyskinesia in Parkinson's Disease.

Authors:
Satoshi Goto

Front Cell Neurosci 2017 21;11:364. Epub 2017 Nov 21.

Department of Neurodegenerative Disorders Research, Institute of Biomedical Sciences, Graduate School of Medical Sciences, Tokushima University, Tokushima, Japan.

The motor symptoms of Parkinson's disease (PD) result from striatal dopamine (DA) deficiency due to a progressive degeneration of nigral dopaminergic cells. Although DA replacement therapy is the mainstay to treat parkinsonian symptoms, a long-term daily administration of levodopa often develops levodopa-induced dyskinesia (LID). LID is closely linked to the dysregulation of cyclic adenosine monophosphate (cAMP) signaling cascades in the medium spiny neurons (MSNs), the principal neurons of the striatum, which are roughly halved with striatonigral MSNs by striatopallidal MSNs. Read More

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http://dx.doi.org/10.3389/fncel.2017.00364DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5696598PMC
November 2017
1 Read

Potential clinical utility of multiple system atrophy biomarkers.

Authors:
Kurt A Jellinger

Expert Rev Neurother 2017 Dec 23;17(12):1189-1208. Epub 2017 Oct 23.

a Institute of Clinical Neurobiology , Vienna , Austria.

Introduction: Multiple system atrophy (MSA), an adult-onset, fatal disorder of uncertain etiology, characterized by parkinsonism, cerebellar, autonomic and motor dysfunctions, is an α-synucleinopathy with glioneuronal degeneration involving multiple parts of the nervous system. The clinical variants correlate with the morphological phenotypes of striatonigral degeneration (MSA-P), olivoponto-cerebellar atrophy (MSA-C), and mixed type MSA. Neuropathological hallmark is the deposition of aberrant α-synuclein in glia and neurons forming cytoplasmic inclusions that cause cell dysfunction/demise. Read More

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http://dx.doi.org/10.1080/14737175.2017.1392239DOI Listing
December 2017
11 Reads

Multiple System Atrophy: An Oligodendroglioneural Synucleinopathy1.

Authors:
Kurt A Jellinger

J Alzheimers Dis 2018 ;62(3):1141-1179

Institute of Clinical Neurobiology, Vienna, Austria.

Multiple system atrophy (MSA) is an orphan, fatal, adult-onset neurodegenerative disorder of uncertain etiology that is clinically characterized by various combinations of parkinsonism, cerebellar, autonomic, and motor dysfunction. MSA is an α-synucleinopathy with specific glioneuronal degeneration involving striatonigral, olivopontocerebellar, and autonomic nervous systems but also other parts of the central and peripheral nervous systems. The major clinical variants correlate with the morphologic phenotypes of striatonigral degeneration (MSA-P) and olivopontocerebellar atrophy (MSA-C). Read More

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http://www.medra.org/servlet/aliasResolver?alias=iospress&am
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http://dx.doi.org/10.3233/JAD-170397DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5870010PMC
January 2018
11 Reads

Evaluation of striatonigral connectivity using probabilistic tractography in Parkinson's disease.

Neuroimage Clin 2017 9;16:557-563. Epub 2017 Sep 9.

William S. Middleton Memorial Veterans Hospital, Madison, WI, USA.

The cardinal movement abnormalities of Parkinson's disease (PD), including tremor, muscle rigidity, and reduced speed and frequency of movements, are caused by degeneration of dopaminergic neurons in the substantia nigra that project to the putamen, compromising information flow through frontal-subcortical circuits. Typically, the nigrostriatal pathway is more severely affected on the side of the brain opposite (contralateral) to the side of the body that manifests initial symptoms. Several studies have suggested that PD is also associated with changes in white matter microstructural integrity. Read More

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http://dx.doi.org/10.1016/j.nicl.2017.09.009DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5608174PMC
June 2018
11 Reads

Recent advances in neuropathology, biomarkers and therapeutic approach of multiple system atrophy.

J Neurol Neurosurg Psychiatry 2018 Feb 31;89(2):175-184. Epub 2017 Aug 31.

Department of Neuroscience, Mayo Clinic, Jacksonville, Florida, USA.

Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterised by a variable combination of autonomic failure, levodopa-unresponsive parkinsonism, cerebellar ataxia and pyramidal symptoms. The pathological hallmark is the oligodendrocytic glial cytoplasmic inclusion (GCI) consisting of α-synuclein; therefore, MSA is included in the category of α-synucleinopathies. MSA has been divided into two clinicopathological subtypes: MSA with predominant parkinsonism and MSA with predominant cerebellar ataxia, which generally correlate with striatonigral degeneration and olivopontocerebellar atrophy, respectively. Read More

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http://dx.doi.org/10.1136/jnnp-2017-315813DOI Listing
February 2018
1 Read

Bilateral striatal necrosis caused by a founder mitochondrial 14459G>A mutation in two independent Japanese families.

J Neurol Sci 2017 Jul 10;378:177-181. Epub 2017 May 10.

Department of Neurology, Gunma University Graduate School of Medicine, 3-39-22 Showa-machi, Maebashi, Gunma 371-8511, Japan. Electronic address:

Bilateral striatal necrosis (BSN) has many causes and is characterized by unique clinical and neuroradiological features. Herein, we report a clinical and genetic analysis of three BSN cases from two independent Japanese families harboring a mitochondrial DNA (mtDNA) 14459G>A mutation located in a coding region of the NADH dehydrogenase subunit 6 gene. In the first family, two male siblings from non-consanguineous parents exhibited similar phenotypes, with infantile-onset generalized dystonia. Read More

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http://dx.doi.org/10.1016/j.jns.2017.05.015DOI Listing
July 2017
9 Reads

Multiple system atrophy: clinicopathological characteristics in Japanese patients.

Proc Jpn Acad Ser B Phys Biol Sci 2017 ;93(5):251-258

Department of Neurology, Brain Research Institute, Niigata University.

Multiple system atrophy (MSA) is an adult-onset neurodegenerative disorder that has both clinical and pathological variants. Clinical examples include MSA with predominant cerebellar ataxia (MSA-C) and MSA with predominant parkinsonism (MSA-P), whereas olivopontocerebellar atrophy and striatonigral degeneration represent pathological variants. We performed systematic reviews of studies that addressed the relative frequencies of clinical or pathological variants of MSA in various populations to determine the clinicopathological characteristics in Japanese MSA. Read More

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http://dx.doi.org/10.2183/pjab.93.016DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5489432PMC
May 2017
6 Reads

Perirhinal accumulation of neuronal alpha-synuclein in a multiple system atrophy patient with dementia.

Neuropathology 2017 Oct 16;37(5):431-440. Epub 2017 Apr 16.

Division of Neurology, Department of Medicine, Nihon University School of Medicine, Tokyo, Japan.

We report the case of a 79-year-old Japanese woman who developed cerebellar ataxia followed by rigidity, dysautonomia and cognitive disorders, and was thus clinically diagnosed as having possible MSA with dementia. Neuropathological findings demonstrated not only olivopontocerebellar and striatonigral degeneration with frequent glial cytoplasmic inclusions (GCIs), but also degenerative changes in the parahippocampal region, accentuated in the anterior portion of perirhinal cortex, where neuronal cytoplasmic inclusions (NCIs) and NFTs were numerous while GCIs were limited. NCIs were frequent in the deep layer, whereas NFTs were more frequent in superficial cortical layers. Read More

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http://dx.doi.org/10.1111/neup.12381DOI Listing
October 2017
19 Reads

Bilateral striatal necrosis caused by ADAR mutations in two siblings with dystonia and freckles-like skin changes that should be differentiated from Leigh syndrome.

Folia Neuropathol 2016 ;54(4):405-409

Department of Medical Genetics, The Children's Memorial Health Institute, Warsaw, Poland.

Pathogenic molecular variants in the ADAR gene are a known cause of rare diseases, autosomal recessive Aicardi- Goutières syndrome type 6, severe infantile encephalopathy with intracranial calcifications and dominant dyschromatosis symmetrica hereditaria, demonstrated mainly in Asian adults. Recently, they have been also found in patients with nonsyndromic bilateral striatal necrosis accompanied by skin changes of the freckles-like type. Here, we present Polish siblings with acute onset and slowly progressive extrapyramidal syndrome with preserved intellectual abilities and basal ganglia changes found in MRI. Read More

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http://www.termedia.pl/doi/10.5114/fn.2016.64819
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http://dx.doi.org/10.5114/fn.2016.64819DOI Listing
May 2017
10 Reads

Early Motor Fluctuations in a Patient with Striatonigral Degeneration.

Case Rep Neurol 2016 Sep-Dec;8(3):243-250. Epub 2016 Dec 13.

Department of Neurology, Tokai University Oiso Hospital, Oiso, Japan.

We report a 44-year-old female with striatonigral degeneration (SND) who showed wearing-off oscillations after 4 months of levodopa treatment. The patient presented with asymmetric left-side dominant rigidity, and levodopa was effective at first. However, she began to show wearing-off oscillations of motor symptoms, which gradually worsened thereafter. Read More

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https://www.karger.com/Article/FullText/453254
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http://dx.doi.org/10.1159/000453254DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC5216213PMC
December 2016
4 Reads

Exome sequencing coupled with mRNA analysis identifies NDUFAF6 as a Leigh gene.

Mol Genet Metab 2016 11 3;119(3):214-222. Epub 2016 Sep 3.

Medical Genetics, University of Siena, Siena, Italy.

We report here the case of a young male who started to show verbal fluency disturbance, clumsiness and gait anomalies at the age of 3.5years and presented bilateral striatal necrosis. Clinically, the diagnosis was compatible with Leigh syndrome but the underlying molecular defect remained elusive even after exome analysis using autosomal/X-linked recessive or de novo models. Read More

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http://dx.doi.org/10.1016/j.ymgme.2016.09.001DOI Listing
November 2016
9 Reads

Lipopolysaccharide-induced functional and structural injury of the mitochondria in the nigrostriatal pathway.

Neurosci Res 2017 Jan 22;114:62-69. Epub 2016 Sep 22.

College of Pharmacy, Yeungnam University, 280 Daehak-Ro, Gyeongsan, Gyeongbuk 38541, Republic of Korea. Electronic address:

Accumulating evidence suggests that chronic inflammation plays a role in the progressive dopaminergic neurodegeneration that occurs in Parkinson's disease. It has been hypothesized that inflammation mediates neuronal damage via exacerbation of a vicious cycle of oxidative stress and mitochondrial dysfunction. The bacterial endotoxin, lipopolysaccharide (LPS), induces microglial activation and inflammation driven dopaminergic neurodegeneration. Read More

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http://dx.doi.org/10.1016/j.neures.2016.09.007DOI Listing
January 2017
6 Reads

Neuroanatomical changes extend beyond striatal atrophy in X-linked dystonia parkinsonism.

Parkinsonism Relat Disord 2016 10 26;31:91-97. Epub 2016 Jul 26.

Department of Neurology, University of Lübeck, Lübeck, Germany.

Background: X-linked dystonia-parkinsonism (XDP) is an inherited neurodegenerative adult-onset basal ganglia model disease associated with severe striatal atrophy. Anatomical changes exceeding striatal pathology were not yet described in XDP. The present study aimed to assess the microstructure of white matter tracts in XDP using magnetic resonance tomography. Read More

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http://dx.doi.org/10.1016/j.parkreldis.2016.07.012DOI Listing
October 2016
4 Reads

Progressive loss of nigrostriatal dopaminergic neurons induced by inflammatory responses to fipronil.

Toxicol Lett 2016 Sep 14;258:36-45. Epub 2016 Jun 14.

Department of Pharmacology, College of Medicine, Hanyang University, Seoul, Republic of Korea; Hanyang Biomedical Research Institute, Seoul, Republic of Korea; Graduate School of Biomedical Science and Engineering, Hanyang University, Seoul, Republic of Korea. Electronic address:

Inflammatory responses are involved in mechanisms of neuronal cell damage in the pathogenesis of neurodegenerative diseases such as Parkinson's disease (PD). We investigated the mechanisms whereby inflammatory responses contribute to loss of dopaminergic neurons in fipronil (FPN)-treated rats. After stereotaxic injection of FPN in the substantia nigra (SN), the number of tyrosine hydroxylase (TH)-positive neurons and the levels of TH expression in the SN decreased at 7days, and a significant decrease was observed at 14days with a subsequent reduction in striatal TH expression. Read More

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http://dx.doi.org/10.1016/j.toxlet.2016.06.011DOI Listing
September 2016
6 Reads

Frequent globular neuronal cytoplasmic inclusions in the medial temporal region as a possible characteristic feature in multiple system atrophy with dementia.

Neuropathology 2016 Oct 11;36(5):421-431. Epub 2016 Mar 11.

Department of Neurology, Tokyo Metropolitan Neurological Hospital, Tokyo Metropolitan Neurological Hospital, Fuchu-shi, Tokyo, Japan.

Multiple system atrophy (MSA) is an adult-onset neurodegenerative disease, which is characterized clinically by parkinsonism, cerebellar ataxia and/or autonomic dysfunction, and pathologically by alpha-synuclein-related multisystem neurodegeneration, so-called alpha-synucleinopathy, which particularly involves the striatonigral and olivopontocerebellar systems, with glial cytoplasmic inclusions and neuronal cytoplasmic/nuclear inclusions (NCIs/NNIs). In the recent consensus criteria for the diagnosis of MSA, dementia is described as one of the features not supporting a diagnosis of MSA. However, MSA with dementia has been reported, although the location of the lesion responsible for the dementia remains unclear. Read More

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http://dx.doi.org/10.1111/neup.12289DOI Listing
October 2016
9 Reads

Coenzyme Q10 Levels Are Decreased in the Cerebellum of Multiple-System Atrophy Patients.

PLoS One 2016 19;11(2):e0149557. Epub 2016 Feb 19.

Department of Molecular Neuroscience, UCL Institute of Neurology, Queen Square, London, United Kingdom.

Background: The objective of this study was to evaluate whether the levels of coenzyme Q10 (CoQ10) in brain tissue of multiple system atrophy (MSA) patients differ from those in elderly controls and in patients with other neurodegenerative diseases.

Methods: Flash frozen brain tissue of a series of 20 pathologically confirmed MSA patients [9 olivopontocerebellar atrophy (OPCA) type, 6 striatonigral degeneration (SND) type, and 5 mixed type] was used for this study. Elderly controls (n = 37) as well as idiopathic Parkinson's disease (n = 7), dementia with Lewy bodies (n = 20), corticobasal degeneration (n = 15) and cerebellar ataxia (n = 18) patients were used as comparison groups. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0149557PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4760984PMC
July 2016
14 Reads

Importance of (123)I-ioflupane SPECT and Myocardial MIBG Scintigraphy to Determine the Candidate of Deep Brain Stimulation for Parkinson's Disease.

Neurol Med Chir (Tokyo) 2016 20;56(3):125-31. Epub 2016 Jan 20.

Departments of Neurosurgery, University of Toyama.

(123)I-ioflupane SPECT (DaTscan) is an examination that detects presynaptic dopamine neuronal dysfunction, and has been used as a diagnostic tool to identify degenerative parkinsonism. Additionally, myocardial (123)I-metaiodobenzyl guanidine (MIBG) scintigraphy measures the concentration of cardiac sympathetic nerve fibers and is used to diagnose Parkinson's disease (PD). These exams are used as adjuncts in the diagnosis of parkinsonism, however, the relationship of these two examinations are not well-known. Read More

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http://dx.doi.org/10.2176/nmc.oa.2015-0234DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4791306PMC
May 2017
17 Reads

Loss of Striatonigral GABAergic Presynaptic Inhibition Enables Motor Sensitization in Parkinsonian Mice.

Neuron 2015 Sep;87(5):976-88

Department of Neurology, Columbia University, New York, NY 10032, USA; Department of Pharmacology, Columbia University, New York, NY 10032, USA; Department of Psychiatry, Columbia University, New York, NY 10032, USA; New York State Psychiatric Institute, New York, NY 10032, USA. Electronic address:

Degeneration of dopamine (DA) neurons in Parkinson's disease (PD) causes hypokinesia, but DA replacement therapy can elicit exaggerated voluntary and involuntary behaviors that have been attributed to enhanced DA receptor sensitivity in striatal projection neurons. Here we reveal that in hemiparkinsonian mice, striatal D1 receptor-expressing medium spiny neurons (MSNs) directly projecting to the substantia nigra reticulata (SNr) lose tonic presynaptic inhibition by GABAB receptors. The absence of presynaptic GABAB response potentiates evoked GABA release from MSN efferents to the SNr and drives motor sensitization. Read More

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http://www.cell.com/neuron/pdf/S0896-6273(15)00716-3.pdf
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http://linkinghub.elsevier.com/retrieve/pii/S089662731500716
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http://dx.doi.org/10.1016/j.neuron.2015.08.022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4559856PMC
September 2015
6 Reads

Lack of additive role of ageing in nigrostriatal neurodegeneration triggered by α-synuclein overexpression.

Acta Neuropathol Commun 2015 Jul 25;3:46. Epub 2015 Jul 25.

University de Bordeaux, Institut des Maladies Neurodégénératives, UMR 5293, 33000, Bordeaux, France.

Introduction: Parkinson's disease (PD) is a progressive neurodegenerative disorder characterized by the loss of dopaminergic neurons as well as the presence of proteinaceous inclusions named Lewy bodies. α-synuclein (α-syn) is a major constituent of Lewy bodies, and the first disease-causing protein characterized in PD. Several α-syn-based animal models of PD have been developed to investigate the pathophysiology of PD, but none of them recapitulate the full picture of the disease. Read More

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http://dx.doi.org/10.1186/s40478-015-0222-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4513748PMC
July 2015
19 Reads

Failure of Neuroprotection Despite Microglial Suppression by Delayed-Start Myeloperoxidase Inhibition in a Model of Advanced Multiple System Atrophy: Clinical Implications.

Neurotox Res 2015 Oct 21;28(3):185-94. Epub 2015 Jul 21.

Department of Neurology, Innsbruck Medical University, Anichstraße 35, 6020, Innsbruck, Austria.

Multiple system atrophy (MSA) is a rapidly progressive neurodegenerative disease. Post-mortem hallmarks of MSA neuropathology include oligodendroglial α-synuclein (αSYN) inclusions, striatonigral degeneration, olivopontocerebellar atrophy, and increased microglial activation that accompanies the wide spread neurodegeneration. Recently, we demonstrated upregulation of myeloperoxidase (MPO) in activated microglia and provided evidence for the role of microglial MPO in the mediation of MSA-like neurodegeneration (Stefanova et al. Read More

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http://dx.doi.org/10.1007/s12640-015-9547-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4556742PMC
October 2015
22 Reads
9 Citations
3.540 Impact Factor

Progression of subcortical atrophy and iron deposition in multiple system atrophy: a comparison between clinical subtypes.

J Neurol 2015 Aug 28;262(8):1876-82. Epub 2015 May 28.

Department of Neurology, Research Institute for Convergence of Biomedical Science and Technology, Pusan National University Yangsan Hospital, Beomo-ri, Mulgum-eup, Yangsan, Gyeongsangnam-do, 626-770, South Korea,

Magnetic resonance imaging (MRI) can be useful not only for the diagnosis of multiple system atrophy (MSA) itself, but also to distinguish between different clinical subtypes. This study aimed to investigate whether there are differences in the progression of subcortical atrophy and iron deposition between two variants of MSA. Two serial MRIs at baseline and follow-up were analyzed in eight patients with the parkinsonian variant MSA (MSA-P), nine patients with cerebellar variant MSA (MSA-C), and fifteen patients with Parkinson's disease (PD). Read More

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http://dx.doi.org/10.1007/s00415-015-7785-5DOI Listing
August 2015
9 Reads

Atypical multiple system atrophy is a new subtype of frontotemporal lobar degeneration: frontotemporal lobar degeneration associated with α-synuclein.

Acta Neuropathol 2015 Jul 12;130(1):93-105. Epub 2015 May 12.

Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL, 32224, USA.

Multiple system atrophy (MSA) is a sporadic neurodegenerative disease clinically characterized by cerebellar signs, parkinsonism, and autonomic dysfunction. Pathologically, MSA is an α-synucleinopathy affecting striatonigral and olivopontocerebellar systems, while neocortical and limbic involvement is usually minimal. In this study, we describe four patients with atypical MSA with clinical features consistent with frontotemporal dementia (FTD), including two with corticobasal syndrome, one with progressive non-fluent aphasia, and one with behavioral variant FTD. Read More

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http://dx.doi.org/10.1007/s00401-015-1442-zDOI Listing
July 2015
4 Reads

Novel fast adapting interneurons mediate cholinergic-induced fast GABAA inhibitory postsynaptic currents in striatal spiny neurons.

Eur J Neurosci 2015 Jul 9;42(2):1764-74. Epub 2015 May 9.

Center for Molecular and Behavioral Neuroscience, Rutgers, the State University of New Jersey, 197 University Avenue, Newark, NJ, 07102, USA.

Previous work suggests that neostriatal cholinergic interneurons control the activity of several classes of GABAergic interneurons through fast nicotinic receptor-mediated synaptic inputs. Although indirect evidence has suggested the existence of several classes of interneurons controlled by this mechanism, only one such cell type, the neuropeptide-Y-expressing neurogliaform neuron, has been identified to date. Here we tested the hypothesis that in addition to the neurogliaform neurons that elicit slow GABAergic inhibitory responses, another interneuron type exists in the striatum that receives strong nicotinic cholinergic input and elicits conventional fast GABAergic synaptic responses in projection neurons. Read More

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http://dx.doi.org/10.1111/ejn.12915DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4510010PMC
July 2015
11 Reads

A rat model of striatonigral degeneration generated by simultaneous injection of 6-hydroxydopamine into the medial forebrain bundle and quinolinic acid into the striatum.

J Korean Med Sci 2014 Nov 4;29(11):1555-61. Epub 2014 Nov 4.

Department of Neurological Surgery, Asan Medical Center, University of Ulsan College of Medicine, Seoul, Korea.

A double toxin-double lesion strategy is well-known to generate a rat model of striatonigral degeneration (SND) such as multiple system atrophy-parkinsonian type. However, with this model it is difficult to distinguish SND from Parkinson's disease (PD). In this study, we propose a new rat model of SND, which is generated by simultaneous injection of 6-hydroxydopamine into the medial forebrain bundle and quinolinic acid into the striatum. Read More

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http://dx.doi.org/10.3346/jkms.2014.29.11.1555DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4234925PMC
November 2014
13 Reads

Neuropathology of multiple system atrophy: new thoughts about pathogenesis.

Authors:
Kurt A Jellinger

Mov Disord 2014 Dec 9;29(14):1720-41. Epub 2014 Oct 9.

Institute of Clinical Neurobiology, Vienna, Austria.

Multiple system atrophy (MSA) is a fatal adult-onset neurodegenerative disorder of uncertain etiology, clinically manifesting with autonomic failure associated with parkinsonism, cerebellar dysfunction, and pyramidal signs in variable combination. The pathological process affects central autonomic, striatonigral, and olivopontocerebellar systems. These show varying degrees of neurodegeneration and underlie the stratification of the heterogenous disorder into MSA-P and MSA-C clinical variants, which correlate to the morphologic phenotypes of striatonigral degeneration and olivopontocerebellar atrophy (MSA-C). Read More

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http://dx.doi.org/10.1002/mds.26052DOI Listing
December 2014
2 Reads

Neuropathologic changes of multiple system atrophy and diffuse Lewy body disease.

Semin Neurol 2014 Apr 25;34(2):210-6. Epub 2014 Jun 25.

Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York, New York.

The accumulation of fibrillar α-synuclein protein is the pathological hallmark occurring in both multiple system atrophy (MSA) and diffuse Lewy body disease (DLBD). The oligodendrocytes are especially involved in MSA, while subtypes of neurons are the targets in DLBD. In both instances, the changes are widespread within the central nervous system, but with distinct and topistic vulnerability. Read More

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https://www.thieme-connect.com/products/ejournals/pdf/10.105
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http://www.thieme-connect.de/DOI/DOI?10.1055/s-0034-1381732
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http://dx.doi.org/10.1055/s-0034-1381732DOI Listing
April 2014
4 Reads

Accuracy of portable polygraphy for the diagnosis of sleep apnea in multiple system atrophy.

Sleep Med 2014 Apr 21;15(4):476-9. Epub 2014 Feb 21.

Université de Bordeaux, Institut des Maladies Neurodégénératives, UMR 5293, Bordeaux, France; CNRS, Institut des Maladies Neurodégénératives, UMR 5293, Bordeaux, France; Department of Clinical Neurophysiology, University Hospital Bordeaux, France.

Objective: To assess the diagnostic accuracy of portable polygraphy (PG) for the detection of sleep apnea (SA) in multiple system atrophy (MSA).

Methods: Thirty consecutive patients with probable MSA underwent PG (overnight recording of nasal flow, thoracic/abdominal movements and pulse oximetry), followed 4 weeks later by full polysomnography (PSG) (reference standard). The accuracy of PG was first assessed using the same threshold as for PSG (apnea-hypopnea index [AHI]≥5), then for all possible AHI thresholds using the area under the receiver operating characteristics (AUROC) curve. Read More

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https://linkinghub.elsevier.com/retrieve/pii/S13899457140007
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http://dx.doi.org/10.1016/j.sleep.2013.12.013DOI Listing
April 2014
3 Reads

Towards translational therapies for multiple system atrophy.

Prog Neurobiol 2014 Jul 2;118:19-35. Epub 2014 Mar 2.

Department of Neurology, Innsbruck Medical University, Anichstraße 35, Innsbruck 6020, Austria. Electronic address:

Multiple system atrophy (MSA) is a fatal adult-onset neurodegenerative disorder of uncertain etiopathogenesis manifesting with autonomic failure, parkinsonism, and ataxia in any combination. The underlying neuropathology affects central autonomic, striatonigral and olivopontocerebellar pathways and it is associated with distinctive glial cytoplasmic inclusions (GCIs, Papp-Lantos bodies) that contain aggregates of α-synuclein. Current treatment options are very limited and mainly focused on symptomatic relief, whereas disease modifying options are lacking. Read More

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http://dx.doi.org/10.1016/j.pneurobio.2014.02.007DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4068324PMC
July 2014
15 Reads
15 Citations
9.992 Impact Factor

An update on the cerebellar subtype of multiple system atrophy.

Cerebellum Ataxias 2014 10;1:14. Epub 2014 Oct 10.

Department of Neurology, Innsbruck Medical University, Anichstrasse 35, A-6020 Innsbruck, Austria.

Multiple system atrophy is a rare and fatal neurodegenerative disorder characterized by progressive autonomic failure, ataxia and parkinsonism in any combination. The clinical manifestations reflect central autonomic and striatonigral degeneration as well as olivopontocerebellar atrophy. Glial cytoplasmic inclusions, composed of α-synuclein and other proteins are considered the cellular hallmark lesion. Read More

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http://dx.doi.org/10.1186/s40673-014-0014-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC4552412PMC
September 2015
14 Reads
4 Citations

Lateralized cortical involvement and contralateral parkinsonism without basal ganglia involvement in two autopsy cases of corticobasal syndrome-Alzheimer's disease.

J Alzheimers Dis 2014 ;40(1):51-5

Department of Pathology, Tokyo Metropolitan Neurological Hospital, Tokyo, Japan Laboratory of Structural Neuropathology, Tokyo Metropolitan Institute for Medical Science, Tokyo, Japan.

Corticobasal syndrome (CBS) is characterized by lateralized motor disturbance due to levodopa nonresponsive parkinsonism and progressive apraxia. Although CBS is neuropathologically heterogeneous, it remains unclear whether the clinical features of all CBS cases are the same. We report two autopsy cases diagnosed clinically as CBS and pathologically as Alzheimer's disease characterized by lateralized cerebral cortical degeneration and absence of significant nigrostriatial lesions. Read More

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http://dx.doi.org/10.3233/JAD-131676DOI Listing
October 2014
7 Reads

A type I interferon signature identifies bilateral striatal necrosis due to mutations in ADAR1.

J Med Genet 2014 Feb 21;51(2):76-82. Epub 2013 Nov 21.

Department of Paediatric Neurology, Leeds General Infirmary, Leeds, UK.

Background: We recently observed mutations in ADAR1 to cause a phenotype of bilateral striatal necrosis (BSN) in a child with the type I interferonopathy Aicardi-Goutières syndrome (AGS). We therefore decided to screen patients with apparently non-syndromic BSN for ADAR1 mutations, and for an upregulation of interferon-stimulated genes (ISGs).

Methods: We performed Sanger sequencing of ADAR1 in a series of patients with BSN presenting to us during our routine clinical practice. Read More

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http://dx.doi.org/10.1136/jmedgenet-2013-102038DOI Listing
February 2014
24 Reads
16 Citations
6.340 Impact Factor

Age-related motor dysfunction and neuropathology in a transgenic mouse model of multiple system atrophy.

Synapse 2014 Mar 15;68(3):98-106. Epub 2013 Nov 15.

Institut des Maladies Neurodégénératives, Université de Bordeaux, UMR 5293, F-33000 Bordeaux, France; CNRS, Institut des Maladies Neurodégénératives, UMR 5293, F-33000 Bordeaux, France.

Multiple system atrophy (MSA) is a neurodegenerative disorder characterized by a progressive degeneration of the striatonigral, olivo-ponto-cerebellar, and autonomic systems. Glial cytoplasmic inclusions (GCIs) containing alpha-synuclein represent the hallmark of MSA and are recapitulated in mice expressing alpha-synuclein in oligodendrocytes. To assess if oligodendroglial expression of human wild-type alpha-synuclein in mice (proteolipid promoter, PLP-SYN) could be associated with age-related deficits, PLP-SYN and wild-type mice were assessed for motor function, brain morphometry, striatal levels of dopamine and metabolites, dopaminergic loss, and distribution of GCIs. Read More

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http://dx.doi.org/10.1002/syn.21719DOI Listing
March 2014
2 Reads

Baseline [(123) I]FP-CIT SPECT (DaTSCAN) severity correlates with medication use at 3 years in Parkinson's disease.

Acta Neurol Scand 2014 Mar 20;129(3):204-8. Epub 2013 Aug 20.

Department of Neurology, Klinikum Bremerhaven, Bremerhaven, Germany.

Background: Presynaptic dopaminergic deficiency on dopamine transporter imaging supports a clinical diagnosis of Parkinson's disease and correlates with the severity of rigidity and bradykinesia. Baseline dopaminergic deficiency predicts clinical severity, but the relationship with subsequent medication use has not been reported.

Methods: A randomly selected cross section of 83 Parkinson's disease (PD) patients who had [(123) I] FP-CIT SPECT at the time of clinical diagnosis was identified. Read More

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http://dx.doi.org/10.1111/ane.12178DOI Listing
March 2014
2 Reads

[Secondary degeneration of the substantia nigra after cerebral infarctions including the striatum].

Brain Nerve 2013 Mar;65(3):289-95

Department of Neurology and Cerebrovascular Medicine, Saitama Medical University International Medical Center, Japan.

Secondary degeneration of the substantia nigra (SN) after damage to the ipsilateral striatum has been widely reported in animal stroke models. Secondary degeneration of the SN frequently occurs in stroke patients with damage to the striatum. Decreased γ-aminobutyric acid in the striatonigral pathway causes disinhibition of the SN pars reticulata, which consequently causes neuronal degeneration there. Read More

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March 2013
9 Reads

Oligodendroglial alpha-synucleinopathy and MSA-like cardiovascular autonomic failure: experimental evidence.

Exp Neurol 2013 Sep 8;247:531-6. Epub 2013 Feb 8.

Division of Neurobiology, Department of Neurology and Neurosurgery, Medical University Innsbruck, Anichstrasse 35, 6020 Innsbruck, Austria.

Multiple system atrophy (MSA) is a fatal, rapidly progressive neurodegenerative disease with limited symptomatic treatment options. Discrimination of MSA from other degenerative disorders crucially depends on the presence of early and severe cardiovascular autonomic failure (CAF). We have previously shown that neuropathologic lesions in the central autonomic nuclei similar to the human disease are present in transgenic MSA mice generated by targeted oligodendroglial overexpression of α-syn using the PLP promoter. Read More

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http://dx.doi.org/10.1016/j.expneurol.2013.02.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3748345PMC
September 2013
15 Reads
20 Citations
4.700 Impact Factor

Imaging beyond the striatonigral dopaminergic system in Parkinson's disease.

Hell J Nucl Med 2012 Sep-Dec;15(3):224-32. Epub 2012 Oct 25.

Third University Department of Neurology, Aristotle University of Thessaloniki, Thessaloniki, Makedonia, Greece.

Parkinson 's disease (PD) is characterized by progressive loss of dopaminergic neurons in the nigrostriatal pathway, but this seems to constitute only part of the whole pathological process of the disease. Accumulating data have documented the concomitant degeneration of other dopaminergic pathways and of the serotonergic, cholinergic and noradrenergic neurotransmitter systems. In addition, pathologic process is not only restricted in the brain, since the spinal cord and the peripheral autonomic nervous system are also affected. Read More

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http://dx.doi.org/10.1967/s002449910058DOI Listing
February 2013
4 Reads

Increased neuronal α-synuclein pathology associates with its accumulation in oligodendrocytes in mice modeling α-synucleinopathies.

PLoS One 2012 15;7(10):e46817. Epub 2012 Oct 15.

Biochemistry and Molecular Biology, IMRIC, The Hebrew University-Hadassah Medical School, Jerusalem, Israel.

Multiple system atrophy (MSA) is a progressive neurodegenerative disorder characterized by striatonigral degeneration and olivo-pontocerebellar atrophy. The histopathological hallmark of MSA is glial cytoplasmic inclusions (GCI) within oligodendrocytes, accompanied by neuronal degeneration. MSA is a synucleinopathy, and α-Synuclein (α-Syn) is the major protein constituent of the GCI. Read More

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http://journals.plos.org/plosone/article?id=10.1371/journal.pone.0046817PLOS
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3471961PMC
March 2013
5 Reads

Sirtuin 2 (SIRT2) enhances 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP)-induced nigrostriatal damage via deacetylating forkhead box O3a (Foxo3a) and activating Bim protein.

J Biol Chem 2012 Sep 16;287(39):32307-11. Epub 2012 Aug 16.

Department of Neuroscience, Tufts University School of Medicine, Boston, Massachusetts 02111, USA.

Sirtuins are NAD-dependent protein deacetylases that were shown to have beneficial effects against age-related diseases. SIRT2 is a strong deacetylase that is highly expressed in brain. It has been associated with neurodegenerative diseases. Read More

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http://dx.doi.org/10.1074/jbc.C112.403048DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3463341PMC
September 2012
4 Reads

Human induced pluripotent stem cells improve stroke outcome and reduce secondary degeneration in the recipient brain.

Cell Transplant 2012 10;21(12):2587-602. Epub 2012 Aug 10.

I-Stem, INSERM UMR861, UEVE, Evry Cedex, France.

Human induced pluripotent stem cells (hiPSCs) are a most appealing source for cell replacement therapy in acute brain lesions. We evaluated the potential of hiPSC therapy in stroke by transplanting hiPSC-derived neural progenitor cells (NPCs) into the postischemic striatum. Grafts received host tyrosine hydroxylase-positive afferents and contained developing interneurons and homotopic GABAergic medium spiny neurons that, with time, sent axons to the host substantia nigra. Read More

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http://dx.doi.org/10.3727/096368912X653228DOI Listing
June 2013
20 Reads