Toward understanding Machado–Joseph disease

M do Carmo Costa, HL Paulson - Progress in neurobiology, 2012 - Elsevier
Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3 (SCA3), is
the most common inherited spinocerebellar ataxia and one of many polyglutamine …

The pathogenic mechanisms of polyglutamine diseases and current therapeutic strategies

PO Bauer, N Nukina - Journal of neurochemistry, 2009 - Wiley Online Library
Expansion of CAG trinucleotide repeat within the coding region of several genes results in
the production of proteins with expanded polyglutamine (PolyQ) stretch. The expression of …

DNA Binding Studies of Novel Copper(II) Complexes Containing l-Tryptophan as Chiral Auxiliary:  In Vitro Antitumor Activity of Cu−Sn2 Complex in Human …

M Chauhan, K Banerjee, F Arjmand - Inorganic chemistry, 2007 - ACS Publications
Novel trinuclear complexes C23H31N6O6CuSn2Cl5 [1], C23H31N6O6CuZr2Cl5 [2],
C23H31N6O6ZnSn2Cl5 [3], and C23H31N6O6ZnZr2Cl5 [4] were synthesized and …

Machado–Joseph disease/spinocerebellar ataxia type 3

H Paulson - Handbook of clinical neurology, 2012 - Elsevier
Abstract Machado–Joseph disease (MJD), also known as spinocerebellar ataxia type 3
(SCA3), may be the most common dominantly inherited ataxia in the world. Here I will review …

Decreased antioxidant enzyme activity and increased mitochondrial DNA damage in cellular models of Machado‐Joseph disease

YC Yu, CL Kuo, WL Cheng, CS Liu… - Journal of …, 2009 - Wiley Online Library
Abstract Machado‐Joseph disease (MJD)/spinocerebellar ataxia type 3 (SCA3) is an
autosomal dominant neurodegenerative disorder caused by polyglutamine expansion in the …

[HTML][HTML] From pathogenesis to novel therapeutics for spinocerebellar ataxia type 3: evading potholes on the way to translation

JD Da Silva, A Teixeira-Castro, P Maciel - Neurotherapeutics, 2019 - Elsevier
Abstract Spinocerebellar ataxia type 3 (SCA3), also known as Machado–Joseph disease
(MJD), is a neurodegenerative disorder caused by a polyglutamine expansion in the ATXN3 …

The role of deubiquitinating enzymes in apoptosis

S Ramakrishna, B Suresh, KH Baek - Cellular and Molecular Life Sciences, 2011 - Springer
It has become apparent that ubiquitination plays a critical role in cell survival and cell death.
In addition, deubiquitinating enzymes (DUBs) have been determined to be highly important …

The protective effect of erinacine A–enriched Hericium erinaceus mycelium ethanol extract on oxidative Stress–Induced neurotoxicity in cell and Drosophila models of …

YL Wu, SC Chen, JC Chang, WY Lin, CC Chen… - Free Radical Biology …, 2023 - Elsevier
Spinocerebellar ataxia type 3 (SCA3), or Machado–Joseph disease (MJD), is an inherited
polyglutamine (polyQ) neurodegenerative disease and the most common form of SCA …

From pathways to targets: understanding the mechanisms behind polyglutamine disease

JJ Weber, AS Sowa, T Binder… - BioMed research …, 2014 - Wiley Online Library
The history of polyglutamine diseases dates back approximately 20 years to the discovery of
a polyglutamine repeat in the androgen receptor of SBMA followed by the identification of …

Polyglutamine-expanded ataxin-3 activates mitochondrial apoptotic pathway by upregulating Bax and downregulating Bcl-xL

AH Chou, TH Yeh, YL Kuo, YC Kao, MJ Jou… - Neurobiology of …, 2006 - Elsevier
Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominant neurodegenerative disease
caused by polyglutamine-expanded ataxin-3. In the present study, we expressed disease …