19 results match your criteria mnd contributes

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Ellagic Acid Resensitizes Gemcitabine-Resistant Bladder Cancer Cells by Inhibiting Epithelial-Mesenchymal Transition and Gemcitabine Transporters.

Cancers (Basel) 2021 Apr 22;13(9). Epub 2021 Apr 22.

Graduate Institute of Life Sciences, National Defense Medical Center, Taipei 114, Taiwan.

Gemcitabine (GCB) resistance is a major issue in bladder cancer chemoresistance, but its underlying mechanism has not been determined. Epithelial-mesenchymal transition (EMT) has been shown to be comprehensively involved in GCB resistance in several other cancer types, but the direct connection between EMT and GCB remains unclear. This study was designed to elucidate the mechanism of EMT-related GCB resistance in bladder cancer and identify a potential phytochemical to modulate drug sensitivity. Read More

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Sensorial Impact and Distribution of 3-Methyl-2,4-nonanedione in Cognacs and Spirits.

J Agric Food Chem 2021 Apr 8;69(15):4509-4517. Epub 2021 Apr 8.

Unité de recherche OEnologie, EA 4577, USC 1366 INRAE, ISVV, Univ. Bordeaux, 33882 Villenave d'Ornon cedex, France.

The aim of this study was to improve our knowledge on the chemical markers of Cognac aromas. We report results concerning the distribution and sensorial impact of 3-methyl-2,4-nonanedione (MND), a well-known compound in aged red wine, reminiscent of anise or "dried fruit", according to its concentration. We assayed first this diketone (solid-phase microextraction (SPME)-gas chromatography (GC)/mass spectrometry (MS), chemical ionization (CI)) in many Cognac samples followed by grappa, brandy, rum, whisky, vodka, and fruit spirits, and concentrations ranged from traces to 11. Read More

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Coexisting Lewy body disease and clinical parkinsonism in amyotrophic lateral sclerosis.

Eur J Neurol 2021 Jul 16;28(7):2192-2199. Epub 2021 Apr 16.

Faculty of Medicine, Health and Human Sciences, School of Biomedical Sciences, Dementia Research Centre, Macquarie University, Sydney, NSW, Australia.

Background: Amyotrophic lateral sclerosis (ALS) is associated with a range of clinical phenotypes and shows progressive degeneration of upper and/or lower motor neurons, and phosphorylated 43 kDa TAR DNA-binding protein (pTDP-43) inclusions in motor and non-motor pathways. Parkinsonian features have been reported in up to 30% of ALS patients, and Lewy bodies, normally associated with Lewy body disease (LBD), have been reported in a small number of ALS cases, with unknown clinical relevance. This study investigates the prevalence of clinically relevant LBD in a prospectively studied ALS cohort to determine whether concomitant pathology contributes to the clinical heterogeneity. Read More

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Autophagy and Motor Neuron Diseases.

Adv Exp Med Biol 2020 ;1207:53-74

Liaoning Provincial Center for Clinical Research on Neurological Diseases, The First Affiliated Hospital, Dalian Medical University, Dalian, China.

Motor neuron diseases (MND) are a group of fatal progressive neurodegenerative diseases, which selectively affect the motor system in the anterior horn of spinal cord, brainstem, cortex and pyramidal tract. Motor neurons could be divided into two groups, which are upper groups in the motor cortex and lower groups in the brain stem and spinal cord. Loss of lower motor neurons leads to muscle weakness, wasting and cramps. Read More

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September 2020

A single systemic inflammatory insult causes acute motor deficits and accelerates disease progression in a mouse model of human tauopathy.

Alzheimers Dement (N Y) 2019 9;5:579-591. Epub 2019 Oct 9.

Centre for Clinical Brain Sciences, University of Edinburgh, Edinburgh, UK.

Introduction: Neuroinflammation, which contributes to neurodegeneration, is a consistent hallmark of dementia. Emerging evidence suggests that systemic inflammation also contributes to disease progression.

Methods: The ability of systemically administered lipopolysaccharide (LPS - 500 μg/kg) to effect acute and chronic behavioural changes in C57BL/6 and P301S tauopathy mice was assessed. Read More

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October 2019

Assessing Inspiratory Muscle Strength for Early Detection of Respiratory Failure in Motor Neuron Disease: Should We Use MIP, SNIP, or Both?

Respiration 2019;98(2):114-124. Epub 2019 Apr 24.

Division of Neurology, Department of Clinical Neurosciences, Geneva University Hospitals, Geneva, Switzerland.

Background: Motor neuron disease (MND) invariably impacts on inspiratory muscle strength leading to respiratory failure. Regular assessment of sniff nasal inspiratory pressure (SNIP) and/or maximal mouth inspiratory pressure (MIP) contributes to early detection of a requirement for ventilatory support.

Objectives: The aim of this study was to compare the feasibility, agreement, and performance of both tests in MND. Read More

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September 2020

Motor neurone disease.

Handb Clin Neurol 2018 ;159:345-357

Brain and Mind Centre, University of Sydney, Sydney, NSW, Australia. Electronic address:

Motor neurone disease (MND) patients exhibit poor gait, balance, and postural control, all of which significantly increases their risk of falling. Falls are frequent in the MND population, and are associated with an increased burden of disease. The complex interplay of both motor and extramotor manifestations in this disease contributes to the heterogeneous and multifactorial causes of such dysfunction. Read More

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TDP-43 interacts with mitochondrial proteins critical for mitophagy and mitochondrial dynamics.

Neurosci Lett 2018 06 30;678:8-15. Epub 2018 Apr 30.

Department of Biological Sciences, Delaware State University, Dover, DE 19901, United States; Delaware Center for Neuroscience Research, Delaware State University, Dover, DE 19901, United States. Electronic address:

Transactive response DNA-binding protein of 43 kDa (TDP-43) functions as a heterogeneous nuclear ribonucleoprotein and is the major pathological protein in frontotemporal lobar degeneration (FTLD) and amyotrophic lateral sclerosis/motor neuron disease (ALS/MND). TDP-43 pathology may also be present as a comorbidity in approximately 20-50% of sporadic Alzheimer's disease cases. In a mouse model of MND, full-length TDP-43 increases association with the mitochondria and blocking the TDP-43/mitochondria interaction ameliorates motor dysfunction. Read More

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KCHO-1, a novel herbal anti-inflammatory compound, attenuates oxidative stress in an animal model of amyotrophic lateral sclerosis.

J Vet Sci 2017 Dec;18(4):487-497

Adult Stem Cell Research Center, College of Veterinary Medicine, Seoul National University, Seoul 08826, Korea.

Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease characterized by selective death of motor neurons in the central nervous system. The main cause of the disease remains elusive, but several mutations have been associated with the disease process. In particular, mutant superoxide dismutase 1 (SOD1) protein causes oxidative stress by activating glia cells and contributes to motor neuron degeneration. Read More

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December 2017

Motor Neuron Syndrome as a New Phenotypic Manifestation of Mutation 9185T>C in Gene MTATP6.

Case Rep Neurol Med 2014 8;2014:701761. Epub 2014 Dec 8.

Neurology Department, Centro Hospitalar de Setúbal, Rua Camilo Castelo Branco, 2910-446 Setúbal, Portugal.

Background. The mutation 9185T>C in ATP6 gene, associated with Leigh syndrome, was reported in only few families. Motor neuron disease (MND), both clinically and electrophysiologically, was not previously described in association with this mutation. Read More

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December 2014

Touching moments: phenomenological sociology and the haptic dimension in the lived experience of motor neurone disease.

Sociol Health Illn 2014 Jul 28;36(6):793-806. Epub 2013 Nov 28.

Health Advancement Research Team, School of Sport & Exercise Science, University of Lincoln, Lincoln, UK.

Currently, there is a relative research lacuna in phenomenological research into the lived experience of motor neurone disease. Based on a sociological research project in the UK, involving 42 participants diagnosed with MND, this article explores the potential of a phenomenological sociology for analysing experiences of this drastically life-limiting neurological disorder. Calls have been made for sociological researchers to analyse more fully and deeply the sensory dimension of the lived body, and this article also contributes to this newly developing body of literature. Read More

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The epigenetic switches for neural development and psychiatric disorders.

J Genet Genomics 2013 Jul 9;40(7):339-46. Epub 2013 May 9.

Department of Neonatology, Children's Hospital of Fudan University, Shanghai 201102, China.

The most remarkable feature of the nervous system is that the development and functions of the brain are largely reshaped by postnatal experiences, in joint with genetic landscapes. The nature vs. nurture argument reminds us that both genetic and epigenetic information is indispensable for the normal function of the brain. Read More

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What wires together dies together: verbs, actions and neurodegeneration in motor neuron disease.

Cortex 2012 Jul 31;48(7):936-44. Epub 2011 Aug 31.

Human Cognitive Neuroscience, Department of Psychology, Edinburgh, UK.

For more than a century the research on Motor Neuron Disease (MND) has been dominated by a tension between the concept of a selective, purely motor degeneration and a growing realisation of the high frequency and importance of cognitive symptoms that can culminate in dementia. The present paper aims at integrating these two, seemingly mutually exclusive interpretations of the disease. It is proposed that the cognitive and motor symptoms in MND are due to the same selective neurodegenerative process, spreading along the lines of functional connections in the nervous system. Read More

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[TDP-43 proteinopathies: ALS and frontotemporal dementias].

Authors:
J Prudlo

Fortschr Neurol Psychiatr 2009 Aug 14;77 Suppl 1:S25-7. Epub 2009 Aug 14.

Universität Rostock, Klinik und Poliklinik für Neurologie, Gehlsheimer Strasse 20, Rostock.

Both, amyotrophic lateral sclerosis (ALS) and frontotemporal lobar degeneration with ubiquitin-positive inclusions (FTLD-U), and their combination (FTLD-U/MND) are principally sporadic diseases that are rarely familial. Cytoplasmic ubiquitinated proteinaceous inclusions in motor and extra-motor neurons are the pathological hallmark of all three forms. In 2006, the TAR DNA-binding protein of 43 kDa (TDP-43) was both identified as the key protein component of the ubiquitinated inclusions and recognised as the key protein of a spectrum of diseases that have since been consolidated as TDP-43 proteinopathies. Read More

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Motor neuron diseases and neurotoxic substances: a possible link?

Chem Biol Interact 2009 Jul 24;180(2):127-30. Epub 2009 Mar 24.

College of Bio-information, Chongqing University of Posts and Telecommunications, Chongqing, PR China.

The motor neuron diseases (MNDs) are a group of related neurodegenerative diseases that cause the relative selective progressive death of motor neurons. Exploring the molecular mechanisms underlying MND phenotypes has been hampered by their multifactorial nature and high incidence of sporadic cases, although genetic factors are considered to play a considerable role at present. However, environmental factors, especial exposure to neurotoxic substances, could induce neurotoxicity with the same phenotypes of specific MNDs. Read More

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Cause of death and clinical grading criteria in a cohort of amyotrophic lateral sclerosis cases undergoing autopsy from the Scottish Motor Neurone Disease Register.

J Neurol Neurosurg Psychiatry 2009 Jan;80(1):84-7

Department of Neurology, Ninewells Hospital and Medical School, Dundee, UK.

Background: The Scottish Motor Neurone Disease Register is a population based register of amyotrophic lateral sclerosis/motor neurone disease (ALS/MND) in Scotland, with high case ascertainment levels.

Objective: To investigate the cause of death by autopsy and assess grading criteria in a cohort of cases of ALS from the Scottish MND Register.

Methods: The records of 44 patients undergoing autopsy were reviewed to determine the cause of death, clinical assessment (El Escorial and modified World Federation of Neurology criteria) during life and neuropathological autopsy findings. Read More

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January 2009

The G59S mutation in p150(glued) causes dysfunction of dynactin in mice.

J Neurosci 2007 Dec;27(51):13982-90

Unit of Transgenesis, Laboratory of Neurogenetics, National Institute on Aging, National Institutes of Health, Bethesda, Maryland 20892, USA.

The G59S missense mutation at the conserved microtubule-binding domain of p150(glued), a major component of dynein/dynactin complex, has been linked to an autosomal dominant form of motor neuron disease (MND). To study how this mutation affects the function of the dynein/dynactin complex and contributes to motor neuron degeneration, we generated p150(glued) G59S knock-in mice. We found that the G59S mutation destabilizes p150(glued) and disrupts the function of dynein/dynactin complex, resulting in early embryonic lethality of homozygous knock-in mice. Read More

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December 2007

Respiratory complications related to bulbar dysfunction in motor neuron disease.

Acta Neurol Scand 2001 Apr;103(4):207-13

University of Wales College of Medicine, Department of Medicine (Neurology), Heath Park, Cardiff, CF4 4XN, United Kingdom.

Bulbar dysfunction resulting from corticobulbar pathway or brainstem neuron degeneration is one of the most important clinical problems encountered in motor neuron disease (MND) and contributes to various respiratory complications which are major causes of morbidity and mortality. Chronic malnutrition as a consequence of bulbar muscle weakness may have a considerable bearing on respiratory muscle function and survival. Abnormalities of the control and strength of the laryngeal and pharyngeal muscles may cause upper airway obstruction increasing resistance to airflow. Read More

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Preoperative localization of parathyroid adenoma in patients with concomitant thyroid nodular disease.

World J Surg 2000 Dec;24(12):1573-8

Department of Medical Biophysics and Nuclear Medicine, Hadassah University Hospital, Jerusalem, Israel.

We have previously demonstrated the role of high-resolution ultrasonography (US) in preoperative localization of parathyroid adenoma in patients with primary hyperparathyroidism (PHPT) and no thyroid abnormalities. The present study prospectively evaluated the possible additional value of 99mTc-sestamibi (MIBI) in patients with PHPT and concomitant multinodular thyroid disease (MND). Patients with PHPT underwent US and MIBI scintigraphy prior to neck exploration. Read More

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December 2000
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