17,658 results match your criteria Huntington Disease


Peripheral Blood Involvement at Staging in Patients With Aggressive Peripheral T-Cell Lymphoma.

Clin Lymphoma Myeloma Leuk 2022 Apr 22. Epub 2022 Apr 22.

Section of Hematology, Department of Medicine, Yale University School of Medicine, New Haven, CT, USA. Electronic address:

Introduction: Peripheral T-Cell Lymphomas (PTCL) are a rare subgroup of lymphomas with a poor outcome.Traditional prognostic measures rely heavily on disease stage, and with the advent of targeted treatment, further stratificationcriteria are needed to guide treatment. To date, the impact of blood involvement at diagnosis on outcomes has not been assessed. Read More

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Predicting clinical scores in Huntington's disease: a lightweight speech test.

J Neurol 2022 May 14. Epub 2022 May 14.

Département d'Études Cognitives, École Normale Supérieure, PSL University, 75005, Paris, France.

Objectives: Using brief samples of speech recordings, we aimed at predicting, through machine learning, the clinical performance in Huntington's Disease (HD), an inherited Neurodegenerative disease (NDD).

Methods: We collected and analyzed 126 samples of audio recordings of both forward and backward counting from 103 Huntington's disease gene carriers [87 manifest and 16 premanifest; mean age 50.6 (SD 11. Read More

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Chronic upper airway and systemic inflammation from copier emitted particles in healthy operators at six Singaporean workplaces.

NanoImpact 2021 Apr 15;22:100325. Epub 2021 May 15.

Center for Nanotechnology and Nanotoxicology, Harvard T.H. Chan School of Public Health, 665 Huntington Avenue, Bldg 1, Rm 1310B, Boston, MA 02115, USA; School of Materials Science and Engineering, Nanyang Technological University, N4.1, #01-21, 50 Nanyang Avenue, 639798, Singapore. Electronic address:

Toner-based printing equipment (TPE), including laser printers and photocopiers, utilize several engineered nanomaterials (ENMs) to improve toner performance. Operation of TPE, which rarely employ any exposure controls, generates high exposures to nanoparticles that contain ENMs and complex organics. Epidemiological literature in copier operators documents respiratory effects, including nasal blockage, cough, excessive sputum, and breathing difficulties, cardiovascular effects, oxidative stress, and inflammation. Read More

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ACU193: An Immunotherapeutic Poised to Test the Amyloid β Oligomer Hypothesis of Alzheimer's Disease.

Front Neurosci 2022 26;16:848215. Epub 2022 Apr 26.

Acumen Pharmaceuticals, Inc., Charlottesville, VA, United States.

Alzheimer's disease (AD) is an age-related neurodegenerative disease that affects 50 million people worldwide, with 10 million new cases occurring each year. The emotional and economic impacts of AD on patients and families are devastating. Approved treatments confer modest improvement in symptoms, and recently one treatment obtained accelerated approval from the United States Food and Drug Administration (FDA) and may have modest disease modifying benefit. Read More

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Differential Expression of Cardiac Fat Long Non-Coding RNAs in Coronary Artery Disease.

FASEB J 2022 May;36 Suppl 1

Biomedical Sciences, Marshall University Joan C. Edwards School of Medicine, Huntington, WV.

Coronary artery disease (CAD) is characterized by the buildup of atherosclerotic plaques within the coronary arteries, leading to serious complications. CAD affects 7.2% and 4. Read More

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Sex-Dependent Changes in Vascular Function in Adult Rats Following Prenatal Exposure to Methamphetamine.

FASEB J 2022 May;36 Suppl 1

Department of Pharmaceutical Sciences, Marshall University School of Pharmacy, Huntington, WV.

Background: Prenatal exposure to central nervous system stimulants such as cocaine, nicotine, and caffeine alters cardiovascular function in adult offspring. Methamphetamine use during pregnancy is associated with negative consequences in the offspring. However, most studies of methamphetamine use during pregnancy have focused on behavioral and neurological outcomes. Read More

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Genetic Predisposition to Type II Diabetes in the Hispanic Population of Orange County in Relation to SNP rs1333051 on the CDKN2 Gene.

FASEB J 2022 May;36 Suppl 1

Vanguard University of Southern California, Irvine, CA.

Thirty-four million individuals cope with diabetes within the United States, 95 percent of which have Type II Diabetes (T2D). Hispanics are particularly affected by diabetes and being the largest minority in the U.S. Read More

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Arvanil, a synthetic capsaicin mimetic, synergizes with Irinotecan to trigger enhanced apoptosis in Cisplatin-resistant Human Lung Cancer.

FASEB J 2022 May;36 Suppl 1

Biomedical Sciences, Joan C. Edwards School of Medicine, Marshall University, Huntington, WV.

Cisplatin-based combination therapy is the first line treatment for human lung cancer patients. Initially, cisplatin shows excellent therapeutic response inducing remission in about 80% of lung cancer patients; however, the tumor inevitably relapses (within a year) and this relapsed tumor is resistant to cisplatin. Patients with platinum-resistant or refractory disease have very limited options, as the only standard chemotherapy with an FDA-approved drug, irinotecan has an objective response rate of approximately 3% and little or no survival benefit. Read More

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Novel pathways of intracellular membrane lipid transport and neurodegenerative diseases.

FASEB J 2022 May;36 Suppl 1

Yale University School of Medicine/Howard Hughes Medical Institute, New Haven, CT.

Membrane lipids move from one compartment to another within cells as part of the membranes of vesicular carriers. However, this mode of lipid traffic is complemented by the action of lipid transport proteins that often act at membrane contact sites. Typically, lipid transport by these proteins is achieved by lipid binding modules that shuttle back and forth between the participating membranes. Read More

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The Correlation Between the SNP rs1049594 and Type 2 Diabetes.

FASEB J 2022 May;36 Suppl 1

Vanguard University of Southern California, Irvine, CA.

Type 2 Diabetes (T2D) is the fifth leading cause of death within the Hispanic population in the United States. Composed of Puerto Ricans, Mexicans, Cubans as well as Central and South Americans, 50% of US Hispanic adults are expected to develop T2D. Diabetes can induce several serious health complications if left untreated such as cardiovascular disease and kidney disease. Read More

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Tumor-Suppressor Role of the Caveolar α1-Na/K-ATPase Signalosome in NASH related Hepatocellular Carcinoma.

FASEB J 2022 May;36 Suppl 1

MIIR/ Dept of Surgery, Marshall University Joan C Edwards School of Medicine, Huntington, WV.

Background: Hepatocellular Carcinoma (HCC) is the second and fastest-growing cause of cancer- related mortality worldwide. In the Western countries, due to the epidemic of obesity, non-alcoholic steatohepatitis (NASH) has become the major cause of HCC. Intriguingly, the molecular mechanisms that underlie the progression of NASH to HCC is still unclear. Read More

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Mechanisms in Chronic Kidney Disease and Associated Cardiovascular Remodeling Contributes to the Progression of Cognitive Impairment in Women.

FASEB J 2022 May;36 Suppl 1

Laboratory of Cardiovascular Science, National Institute on Aging, National Institute of Health, Baltimore, MD.

Introduction: In end-stage chronic kidney disease (CKD) (stages 4-5) the prevalence of cognitive impairment (CI) has been estimated at 30-60%, twice the values observed in age- and sex-matched controls. CKD is greater in females vs. males due to a higher risk of renal infections, especially during the pregnancy, which may result in the renal complications later in life, when the level of protective estrogen declines. Read More

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Vaccinating across the aisle: using co-partisan source cues to encourage COVID-19 vaccine uptake in the ideological right.

J Behav Med 2022 May 11. Epub 2022 May 11.

Department of Health Policy and Management, School of Public Health, Texas A&M University, 212 Adriance Lab Rd. 1266 TAMU, College Station, TX, USA.

Overcoming the COVID-19 pandemic in the United States will require most Americans to vaccinate against the disease. However, considerable research suggests that a significant proportion of Americans intend to forego vaccination, putting pandemic recovery at risk. Republicans are one of the largest groups of COVID-19 vaccine hesitant individuals. Read More

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FAN1 nuclease helps to delay Huntington disease.

Authors:
Heather Wood

Nat Rev Neurol 2022 May 9. Epub 2022 May 9.

Nature Reviews Neurology, .

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Ensemble clustering of longitudinal bivariate HIV biomarker profiles to group patients by patterns of disease progression.

Int J Data Sci Anal 2022 May 4:1-14. Epub 2022 May 4.

Department of Biostatistics, Harvard T. H. Chan School of Public Health, 677 Huntington Avenue, Boston, MA 02115 USA.

This paper describes an ensemble cluster analysis of bivariate profiles of HIV biomarkers, viral load and CD4 cell counts, which jointly measure disease progression. Data are from a prevalent cohort of HIV positive participants in a clinical trial of vitamin supplementation in Botswana. These individuals were HIV positive upon enrollment, but with unknown times of infection. Read More

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Beyond the clinical context: the process of losing oneself living with Huntington's disease.

Orphanet J Rare Dis 2022 May 7;17(1):184. Epub 2022 May 7.

Bio3Science Research Network, Belonging to Geography and Urban Studies, Temple University, Philadelphia, USA.

Background: People with Huntington's disease (HD) have increased functional and cognitive dependence. While numerous clinical, genetic, and therapeutic management studies have been carried out, few studies have investigated the disease from the personal experience and the context of people living with HD. To better serve these patients, our purpose is to understand, from the perspective of the patient and their families, how people with HD cope with their daily lives outside the clinical setting. Read More

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Crescendo Angina Secondary to Congenital Absence of the Left Circumflex Coronary Artery: A Case Report.

Cureus 2022 Apr 1;14(4):e23749. Epub 2022 Apr 1.

Cardiovascular Medicine, St. Mary's Medical Center, Huntington, USA.

Congenital coronary artery abnormalities (CAAs) are very rare in the general population. Among these congenital anomalies, left circumflex artery (LCx) anomaly is the most common. Although many are asymptomatic, a small percentage of patients with this anomaly present with angina-like symptoms. Read More

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No optical coherence tomography changes in premanifest Huntington's disease mutation carriers far from disease onset.

Brain Behav 2022 May 5:e2592. Epub 2022 May 5.

Department of Neurology, Inselspital, Bern University Hospital and University of Bern, Bern, Switzerland.

Background: Spectral-domain optical coherence tomography (OCT) may detect retinal changes as a biomarker in neurodegenerative diseases like manifest Huntington's disease (HD). We investigate macular retinal layer thicknesses in a premanifest HD (pre-HD) cohort and healthy controls (HC).

Methods: Pre-HD mutation carriers underwent standardized ratings and a preset macular OCT scan. Read More

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Nucleoporin POM121 signals TFEB-mediated autophagy via activation of SIGMAR1/sigma-1 receptor chaperone by pridopidine.

Autophagy 2022 May 4:1-26. Epub 2022 May 4.

Cellular Pathobiology Section, Integrative Neuroscience Research Branch, Intramural Research Program, National Institute on Drug Abuse, National Institutes of Health, DHHS, 333 Cassell Drive, Baltimore, Maryland 21224, USA.

Macroautophagy/autophagy is an essential process for cellular survival and is implicated in many diseases. A critical step in autophagy is the transport of the transcription factor TFEB from the cytosol into the nucleus, through the nuclear pore (NP) by KPNB1/importinβ1. In the C9orf72 subtype of amyotrophic lateral sclerosis-frontotemporal lobar degeneration (ALS-FTD), the hexanucleotide (G4C2)RNA expansion (HRE) disrupts the nucleocytoplasmic transport of TFEB, compromising autophagy. Read More

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Disordered Decision Making: A Cognitive Framework for Apathy and Impulsivity in Huntington's Disease.

Mov Disord 2022 May 2. Epub 2022 May 2.

Department of Medicine, University of Otago, Christchurch, New Zealand.

A caregiver's all-too-familiar narrative - "He doesn't think through what he does, but mostly he does nothing." Apathy and impulsivity, debilitating and poorly understood, commonly co-occur in Huntington's disease (HD). HD is a neurodegenerative disease with manifestations bridging clinical neurology and psychiatry. Read More

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What did we learn from neural grafts in Huntington disease?

Rev Neurol (Paris) 2022 Apr 28. Epub 2022 Apr 28.

Département d'études cognitives, école normale supérieure, PSL University, 75005 Paris, France; Inserm U955, Institut Mondor de Recherche Biomédicale, Equipe E01 NeuroPsychologie Interventionnelle, 94000 Créteil, France; Faculté de médecine, Université Paris-Est Créteil, 94000 Créteil, France; Assistance Publique-Hôpitaux de Paris, National Reference Center for Huntington's Disease, Neurology Department, Henri Mondor-Albert Chenevier Hospital, Créteil, France. Electronic address:

Huntington's disease is a rare, severe, and inherited neurodegenerative disorder that affects young adults. To date, there is no treatment to stop its progression. The primary atrophy of the striatum in HD, is limited in space and centrally focalised in the brain and thus constitutes a good candidate for graft. Read More

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Nuclear pore complexes - a doorway to neural injury in neurodegeneration.

Nat Rev Neurol 2022 Apr 29. Epub 2022 Apr 29.

Brain Science Institute, Johns Hopkins University School of Medicine, Baltimore, MD, USA.

The genetic underpinnings and end-stage pathological hallmarks of neurodegenerative diseases are increasingly well defined, but the cellular pathophysiology of disease initiation and propagation remains poorly understood, especially in sporadic forms of these diseases. Altered nucleocytoplasmic transport is emerging as a prominent pathomechanism of multiple neurodegenerative diseases, including amyotrophic lateral sclerosis, Alzheimer disease, frontotemporal dementia and Huntington disease. The nuclear pore complex (NPC) and interactions between its individual nucleoporin components and nuclear transport receptors regulate nucleocytoplasmic transport, as well as genome organization and gene expression. Read More

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Huntington's Disease Progression and Caregiver Burden.

Eur Neurol 2022 Apr 28:1-6. Epub 2022 Apr 28.

Department of Public Health, Poznan University of Medical Sciences, Poznan, Poland.

Huntington's disease (HD) is a neurodegenerative, progressive disorder conditioned by a mutation in the HTT gene. Its progression is dependent on the causative mutation extension. Caregivers of individuals affected by HD, most often patients' relatives, are burdened with the care. Read More

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Cognitive Dysfunction in Repeat Expansion Diseases: A Review.

Front Aging Neurosci 2022 11;14:841711. Epub 2022 Apr 11.

Department of Neurology, Xiangya Hospital, Central South University, Changsha, China.

With the development of the sequencing technique, more than 40 repeat expansion diseases (REDs) have been identified during the past two decades. Moreover, the clinical features of these diseases show some commonality, and the nervous system, especially the cognitive function was affected in part by these diseases. However, the specific cognitive domains impaired in different diseases were inconsistent. Read More

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Neuroprotective effect of CTK 01512-2 recombinant toxin at the spinal cord in a model of Huntington's disease.

Exp Physiol 2022 Apr 27. Epub 2022 Apr 27.

Laboratório de Biologia da Neurotransmissão, Departamento de Morfologia.

New Findings: What is the central question of this study? We investigated the action of intrathecal administration of a novel toxin (CTK01512-2) in a mouse model for Huntington´s disease (HD). We asked if spinal cord neurons can represent a therapeutic target, as the spinal cord seems to be involved in HD motor-symptoms. Pharmacological approaches focusing on the spinal cord and skeletal muscles might represent a more feasible strategy. Read More

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Inosine attenuates 3-nitropropionic acid-induced Huntington's disease-like symptoms in rats via the activation of the A2AR/BDNF/TrKB/ERK/CREB signaling pathway.

Life Sci 2022 Jul 23;300:120569. Epub 2022 Apr 23.

Department of Pharmacology and Toxicology, Faculty of Pharmacy, Cairo University, Egypt.

Huntington's disease (HD) is an autosomal dominant inherited neurodegenerative disease characterized by involuntary bizarre movements, psychiatric symptoms, dementia, and early death. Several studies suggested neuroprotective activities of inosine; however its role in HD is yet to be elucidated. The current study aimed to demonstrate the neuroprotective effect of inosine in 3-nitropropionic acid (3-NP)-induced neurotoxicity in rats while investigating possible underlying mechanisms. Read More

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Early metabolic impairment as a contributor to neurodegenerative disease: Mechanisms and potential pharmacological intervention.

Obesity (Silver Spring) 2022 May;30(5):982-993

Department of Pharmacology and Toxicology, Faculty of Medicine, American University of Beirut, Beirut, Lebanon.

The metabolic syndrome comprises a family of clinical and laboratory findings, including insulin resistance, hyperglycemia, hypertriglyceridemia, low high-density lipoprotein cholesterol levels, and hypertension, in addition to central obesity. The syndrome confers a high risk of cardiovascular mortality. Indeed, metabolic dysfunction has been shown to cause a direct insult to smooth muscle and endothelial components of the vasculature, which leads to vascular dysfunction and hyperreactivity. Read More

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