Results 91 to 100 of about 44,176 (282)

SIRT1 Activity Is Linked to Its Brain Region-Specific Phosphorylation and Is Impaired in Huntington’s Disease Mice [PDF]

, 2015
Huntingtons disease (HD) is a neurodegenerative disorder for which there are no disease-modifying treatments. SIRT1 is a NAD+-dependent protein deacetylase that is implicated in maintaining neuronal health during development, differentiation and ageing ...
Bates, GP, Benjamin, AC, Carnemolla, A, Chini, EN, Jolinon, N, Smith, DL, Tulino, R   +6 more
core   +1 more source

Development and Validation of a Brief Fall Questionnaire (FALL‐HD) for Patients with Huntington's Disease

Movement Disorders Clinical Practice, EarlyView.
ABSTRACT Background Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder caused by a mutation in the huntingtin gene on chromosome 4, leading to progressive cognitive decline, motor impairment, and functional disability. Falls represent the leading cause of nursing home placement in HD.
Japleen Kaur, Deng‐Yuan Liou, Krisha Bagga, Nadeen Youhanan, Andrew Hall, Paul E. Gilbert, Daniel J. Goble, Jody Corey‐Bloom   +7 more
wiley   +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

Emerging Therapies for Huntington’s Disease – Focus on N-Terminal Huntingtin and Huntingtin Exon 1

Biologics: Targets & Therapy, 2022
M Leontien van der Bent, Melvin M Evers, Astrid Vallès uniQure biopharma B.V., Department of Research and Development, Amsterdam, the NetherlandsCorrespondence: Astrid Vallès, uniQure biopharma B.V, Postbus 22506, Amsterdam, 1100 DA, the Netherlands, Tel
van der Bent ML, Evers MM, Vallès A
doaj  

Modulation of Huntingtin Toxicity by BAG1 is Dependent on an Intact BAG Domain

Molecules, 2010
Huntington´s disease, one of the so-called poly-glutamine diseases, is a dominantly inherited movement disorder characterized by formation of cytosolic and nuclear inclusion bodies and progressive neurodegeneration.
Mathias Bähr, Pawel Kermer, Sebastian Deeg, Christoph P. Dohm, Kamila Sroka, Jan Liman   +5 more
doaj   +1 more source

Phase Transition of Huntingtin: Factors and Pathological Relevance

Frontiers in Genetics, 2020
Formation of intracellular mutant Huntingtin (mHtt) aggregates is a hallmark of Huntington’s disease (HD). The mechanisms underlying mHtt aggregation, however, are still not fully understood. A few recent studies indicated mHtt undergoes phase transition,
Junsheng Yang, Xiaotong Yang
doaj   +1 more source

Brainiac Caspases: Beyond the Wall of Apoptosis [PDF]

, 2019
For the last two decades, caspases, a family of cysteine-aspartic proteases, have evolved from being considered solely as regulators of apoptosis or inflammation to having a wider range of functions.
Alonso Bellido, Isabel María, Burguillos García, Miguel Ángel, Espinosa Oliva, Ana María, García Revilla, Juan   +3 more
core   +1 more source

Nuclear Relocation of Normal Huntingtin [PDF]

Traffic, 2001
In Huntington's Disease (HD), the huntingtin protein (Htt) includes an expanded polyglutamine domain. Since mutant Htt concentrates in the nucleus of affected neurons, we have inquired whether normal Htt (Q16−23) is also able to access the nucleus. We observe that a major pool of normal full‐length Htt of HeLa cells is anchored to endosomes and also ...
T, Tao, A M, Tartakoff
openaire   +2 more sources

Interplay Between Sex and Cytosine‐Adenine‐Guanine‐Age Product Score in Huntington's Disease: Clinical and Neuroimaging Perspectives

Movement Disorders, EarlyView.
Abstract Background Huntington's disease (HD) is a progressive neurodegenerative disorder caused by a cytosine‐adenine‐guanine (CAG) repeat expansion in the huntingtin gene. The disease exhibits sex‐related differences in symptomatology and disease progression, but the effect on brain structural biomarkers and the interaction between sex and CAG‐age ...
Jingwen Yao, Grayson Feng, Guowen Shao, Zexi Wang, Adys Mendizabal, Donatello Telesca, Kaiying Fang, Lola Ibragimova, Juwairia Shoaib, Melanie A. Morrison, Janine M. Lupo   +10 more
wiley   +1 more source

Renovating Neural Networks With Viral‐Mediated Gene Transfer From A Tissue Contacting Matrix Mimic

Small, EarlyView.
Fmoc‐DDIKVAV self‐assembling peptidemediated delivery of AAV‐BDNF significantly enhances neuroprotection in the striatum of a mouse model compared with AAV‐BDNF injection alone. This synergistic integration of biomaterial scaffolding and gene therapy holds substantial promise for slowing the progression of neurodegenerative diseases such as Huntington ...
Shiva Soltani Dehnavi, Negar Mahmoudi, Yi Wang, Samuel Cheeseman, Rita Ferreira, Ross D. Hannan, Leszek Lisowski, Vincent S. J. Craig, Niamh Moriarty, Clare L. Parish, Richard J. Williams, David R. Nisbet   +11 more
wiley   +1 more source