Results 51 to 60 of about 16,136 (210)

miR-1 coordinately regulates lysosomal v-ATPase and biogenesis to impact proteotoxicity and muscle function during aging

eLife, 2021
Muscle function relies on the precise architecture of dynamic contractile elements, which must be fine-tuned to maintain motility throughout life. Muscle is also plastic, and remodeled in response to stress, growth, neural and metabolic inputs.
Isabelle Schiffer, Birgit Gerisch, Kazuto Kawamura, Raymond Laboy, Jennifer Hewitt, Martin Sebastian Denzel, Marcelo A Mori, Siva Vanapalli, Yidong Shen, Orsolya Symmons, Adam Antebi   +10 more
doaj   +1 more source

Mitochondria and the Actin Cytoskeleton in Neurodegeneration

Cytoskeleton, EarlyView.
ABSTRACT Mitochondrial dysfunction and cytoskeletal disorganization are widely recognized hallmarks of neurodegenerative diseases such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), and amyotrophic lateral sclerosis (ALS).
Shivani Tuli, Preet Patel, Aneri Shethji, David Gau   +3 more
wiley   +1 more source

The Ubiquitin-Proteasome System in Huntington’s Disease: Are Proteasomes Impaired, Initiators of Disease, or Coming to the Rescue?

Biochemistry Research International, 2012
Huntington’s disease is a progressive neurodegenerative disease, caused by a polyglutamine expansion in the huntingtin protein. A prominent hallmark of the disease is the presence of intracellular aggregates initiated by N-terminal huntingtin fragments ...
Sabine Schipper-Krom, Katrin Juenemann, Eric A. J. Reits   +2 more
doaj   +1 more source

Stem cell models of polyglutamine diseases and their use in cell-based therapies

Frontiers in Neuroscience, 2015
Polyglutamine diseases are fatal neurological disorders that affect the central nervous system. They are caused by mutations in disease genes that contain CAG trinucleotide expansions in their coding regions.
Evangelia eSiska, George eKoliakos, George eKoliakos, Spyros ePetrakis   +3 more
doaj   +1 more source

Alogliptin Reduces Oxidative Stress in Cardiomyocytes and Ameliorates Diabetic Cardiomyopathy via the AURKB/NLGN2 Signaling

The Kaohsiung Journal of Medical Sciences, EarlyView.
ABSTRACT Diabetic cardiomyopathy (DCM) is a common complication of diabetes mellitus. This study investigated the effects of alogliptin on DCM and its underlying mechanisms. A DCM model was constructed and treated with alogliptin. Downstream targets of alogliptin were screened using bioinformatics analysis.
Li‐Jing Jiao, Jing Zhou, Jing Wang, Si‐Nan Zhao, Zhan‐Sheng Zhao, Qian Wang, Ya‐Nan Xie, Wei Jiao   +7 more
wiley   +1 more source

Polyglutamine Repeats in Neurodegenerative Diseases [PDF]

Annual Review of Pathology: Mechanisms of Disease, 2019
Among the age-dependent protein aggregation disorders, nine neurodegenerative diseases are caused by expansions of CAG repeats encoding polyglutamine (polyQ) tracts. We review the clinical, pathological, and biological features of these inherited disorders.
Andrew P, Lieberman, Vikram G, Shakkottai, Roger L, Albin   +2 more
openaire   +2 more sources

Hunting for the cause: Evidence for prion-like mechanisms in Huntington’s disease

Frontiers in Neuroscience, 2022
The hypothesis that pathogenic protein aggregates associated with neurodegenerative diseases spread from cell-to-cell in the brain in a manner akin to infectious prions has gained substantial momentum due to an explosion of research in the past 10–15 ...
Kirby M. Donnelly, Cevannah M. Coleman, Madison L. Fuller, Victoria L. Reed, Dayna Smerina, David S. Tomlinson, Margaret M. Panning Pearce, Margaret M. Panning Pearce   +7 more
doaj   +1 more source

CAG RNAs induce DNA damage and apoptosis by silencing NUDT16 expression in polyglutamine degeneration [PDF]

, 2021
Shaohong Peng, Pei Guo, Xiao Lin, Ying An, Kong Hung Sze, Matthew Ho Yan Lau, Zhefan Stephen Chen, Qianwen Wang, Wen Li, Jacquelyne Ka‐Li Sun, Sum Yi, Ting‐Fung Chan, Kwok‐Fai Lau, Jacky Chi Ki Ngo, Kin Ming Kwan, Chun‐Ho Wong, Sik Lok Lam, Steven C. Zimmerman, Tiziano Tuccinardi, Zhong Zuo, Ho Yu Au‐Yeung, Hei‐Man Chow, Ho Yin Edwin Chan   +22 more
openalex   +1 more source

Cerebrospinal Fluid Proenkephalin Predicts Striatal Atrophy Decades before Clinical Motor Diagnosis in Huntington's Disease

Movement Disorders, EarlyView.
Abstract Background Huntington's disease (HD) is characterized by early, selective, progressive vulnerability of striatal medium spiny neurons (MSNs). Proenkephalin (PENK), a precursor of opioid peptides abundantly expressed in MSNs, is a promising biomarker of striatal integrity, but region‐specific associations and its potential for early‐stage ...
Mena Farag, Michael J. Murphy, Nicola Z. Hobbs, Michela Leocadi, Kate Fayer, Olivia Thackeray, Johan Gobom, Marc Ciosi, Amanda Heslegrave, Henrik Zetterberg, Douglas R. Langbehn, Darren G. Monckton, Edward J. Wild, Sarah J. Tabrizi, Rachael I. Scahill   +14 more
wiley   +1 more source

Sleep Alterations in a Mouse Model of Spinocerebellar Ataxia Type 3

Cells, 2022
Spinocerebellar ataxia type 3 (SCA3) is a neurodegenerative disorder showing progressive neuronal loss in several brain areas and a broad spectrum of motor and non-motor symptoms, including ataxia and altered sleep.
Maria-Efstratia Tsimpanouli, Anjesh Ghimire, Anna J. Barget, Ridge Weston, Henry L. Paulson, Maria do Carmo Costa, Brendon O. Watson   +6 more
doaj   +1 more source