Results 31 to 40 of about 6,842,722 (107)

Modulation of the Stress Granule Component Carhsp1 Mitigates Disease‐Associated Deficits in Spinocerebellar Ataxia Type 3 Mouse Models

Movement Disorders, Volume 41, Issue 6, Page 1516-1527, June 2026.
Abstract Background Spinocerebellar ataxia type 3 (SCA3) is a polyglutamine (polyQ) neurogenerative disorder that results from CAG trinucleotide repeat expansions in the ATXN3 gene, leading to toxic protein aggregate formation and cellular pathway dysfunction.
Tiago Moreira‐Gomes, Adriana Marcelo, Ana Teresa Rajado, Rafael G. Costa, Ricardo Afonso‐Reis, Cristiana R. Madeira, Inês T. Afonso, David V.C. Brito, Adriana A. Vaz, Clévio Nóbrega   +9 more
wiley   +1 more source

Redox environment modulates aggregation of ataxin‐3 in vitro — Implications for drug screening of cysteine‐rich proteins

The FEBS Journal, Volume 293, Issue 11, Page 3376-3398, June 2026.
Redox environment modulates in vitro aggregation of Ataxin‐3, the protein implicated in spinocerebellar ataxia type 3. Reducing conditions stabilize native monomers and prevent aggregation, whereas oxidative conditions promote the formation of non‐native conformers and disulfide‐linked oligomers within the Josephin domain (JD).
Martyna Podlasiak, Martina Sollazzo, Elisa Monaca, Raffaele Sabbatella, Maria Agnese Morando, Oleg Chertkov, Laura Puglisi, Martina Mascellino, Anna Fricano, Sandra Macedo‐Ribeiro, Caterina Alfano   +10 more
wiley   +1 more source

Therapy speech impact in quality of life in patients with Machado-Joseph disease [PDF]

, 2016
A doença de Machado-Joseph é uma doença degenerativa e atualmente considerada a ataxia espinocerebelar mais frequente. O processo degenerativo da doença afeta diferentes regiões e funções do sistema nervoso central e/ou periférico.
Finard, Simone Augusta, Olchik, Maira Rozenfeld, Silva, Bibiana Fuzer da   +2 more
core   +1 more source

Development and Psychometric Evaluation of the Forensic Nursing Competency Scale‐Short Form for Hospital Nurses

Journal of Advanced Nursing, Volume 82, Issue 6, Page 5937-5950, June 2026.
ABSTRACT Aim To develop and evaluate the psychometric properties of the Forensic Nursing Competency Scale‐Short Form (FNCS‐SF) for hospital nurses. Background Nurses who care for victims of sexual abuse, domestic violence and elder or child abuse require forensic nursing competencies.
Youn‐Jung Son, Na Young Jo, Yun Mi Lee, So Hyun Park   +3 more
wiley   +1 more source

Engineering exosomal cargo loading via endogenous molecular pathways: Strategies to enhance therapeutic potential

Interdisciplinary Medicine, Volume 4, Issue 3, May 2026.
This review illustrates how scientists engineer exosomes by hijacking the cell's own cargo‐sorting machinery. These strategies efficiently load therapeutic molecules into natural vesicles, creating powerful next‐generation drug delivery systems (Created with BioGDP.com).
Huanrong Zhu, Chenning Zhang, Shihan Ma, Zhanxue Xu, Cailing Liang, Fang Cheng, Hongbo Chen   +6 more
wiley   +1 more source

Spinal cord damage in Machado-Joseph disease

, 2015
Machado-Joseph disease (SCA3) is the most frequent spinocerebellar ataxia worldwide and characterized by remarkable phenotypic heterogeneity. MRI-based studies in SCA3 focused in the cerebellum and connections, but little is known about cord damage in ...
Lopes-Cendes, Iscia, Bergo, F.P.G., Franca, M.C., Fahl, Camila N, Bergo, Felipe P G, Branco, Lucas Melo T, França, Marcondes C, D'Abreu, A., Lopes-Cendes, I., Fahl, C.N., D'Abreu, Anelyssa, Branco, L.M.T.   +11 more
core   +1 more source

Association Between Cerebellar Metabolic Markers and Activities of Daily Living in Patients With Spinocerebellar Ataxia Type 3

Molecular Genetics &Genomic Medicine, Volume 14, Issue 4, April 2026.
Significant correlations were found between cerebellar metabolite ratios and Activities of Daily Living (ADL) in SCA3 patients. These findings suggest that cerebellar metabolite ratios may serve as potential neuroimaging biomarkers to predict functional disability in SCA3.
Mei Ye, Xiao Ping Qiu, Shengnan Zhang, Ling Wang, Zhongming Gao, Mengyu Liu, Yaping Feng   +6 more
wiley   +1 more source

Sleep Symptoms And Their Clinical Correlates In Machado-joseph Disease

, 2015
Objective - : To evaluate the presence of sleep symptoms in Machado-Joseph disease/spinocerebellar ataxia type 3 (MJD/SCA3). Subjects/methods - : We used a sleep questionnaire and the Epworth Sleepiness Scale to compare 53 patients with MJD/SCA3 and 106 ...
D'Abreu A., Friedman J.H., Cendes F., Conz L., Franca Jr. M., Nucci A.M., Lopes-Cendes I.   +6 more
core   +1 more source

Esophageal achalasia associated with Machado-Joseph disease

, 2004
Esophageal achalasia associated with Machado-Joseph ...
Mike Ewing (13084554), MM Kline (15916742)   +1 more
core   +1 more source

Silencing mutant ataxin-3 rescues motor deficits and neuropathology in Machado-Joseph disease transgenic mice. [PDF]

, 2013
Machado-Joseph disease (MJD) or spinocerebellar ataxia type 3 (SCA3) is an autosomal dominantly-inherited neurodegenerative disorder caused by the over-repetition of a CAG codon in the MJD1 gene.
Isabel Nascimento-Ferreira, Nascimento-Ferreira, Isabel, Clévio Nóbrega, Albuquerque, David, Nicole Déglon (46541), Hirokazu Hirai, David Albuquerque (275749), Onofre, Isabel, Isabel Onofre (275748), Luís Pereira de Almeida, David Albuquerque, Isabel Onofre, Nicole Déglon, Almeida, Luís Pereira de, Hirai, Hirokazu, Déglon, Nicole, Hirokazu Hirai (115426), Isabel Nascimento-Ferreira (275747), Clévio Nóbrega (275746), Luís Pereira de Almeida (221526), Nóbrega, Clévio   +20 more
core   +1 more source