Protein homeostasis in models of aging and age-related conformational disease
T he stability of the proteome is crucial to the health of the cell, and contributes significantly
to the lifespan of the organism. Aging and many age-related diseases have in common the …
to the lifespan of the organism. Aging and many age-related diseases have in common the …
Polyglutamine diseases: the special case of ataxin-3 and Machado–Joseph disease
Polyglutamine (polyQ) diseases are a group of nine neurodegenerative disorders caused by
an unstable CAG expansion in the codifying region of their respective associated genes …
an unstable CAG expansion in the codifying region of their respective associated genes …
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 …
autosomal dominant neurodegenerative disorder caused by polyglutamine expansion in the …
Blood transcriptome sequencing identifies biomarkers able to track disease stages in spinocerebellar ataxia type 3
M Raposo, J Hübener-Schmid, AF Ferreira… - Brain, 2023 - academic.oup.com
Transcriptional dysregulation has been described in spinocerebellar ataxia type 3/Machado-
Joseph disease (SCA3/MJD), an autosomal dominant ataxia caused by a polyglutamine …
Joseph disease (SCA3/MJD), an autosomal dominant ataxia caused by a polyglutamine …
Protective effects of heat shock protein 27 in a model of ALS occur in the early stages of disease progression
PS Sharp, MT Akbar, S Bouri, A Senda, K Joshi… - Neurobiology of …, 2008 - Elsevier
Amyotrophic lateral sclerosis (ALS) is a fatal neuromuscular disorder, characterised by
progressive motor neuron degeneration and muscle paralysis. Heat shock proteins (HSPs) …
progressive motor neuron degeneration and muscle paralysis. Heat shock proteins (HSPs) …
Toward therapeutic targets for SCA3: Insight into the role of Machado–Joseph disease protein ataxin-3 in misfolded proteins clearance
X Li, H Liu, PL Fischhaber, TS Tang - Progress in neurobiology, 2015 - Elsevier
Machado–Joseph disease (MJD, also known as spinocerebellar ataxia type 3, SCA3), an
autosomal dominant neurological disorder, is caused by an abnormal expanded …
autosomal dominant neurological disorder, is caused by an abnormal expanded …
Carbonic anhydrase related proteins: molecular biology and evolution
The catalytically inactive isoforms of α-carbonic anhydrases are known as carbonic
anhydrase related proteins (CARPs). The CARPs occur independently or as domains of …
anhydrase related proteins (CARPs). The CARPs occur independently or as domains of …
Decrease of Hsp25 protein expression precedes degeneration of motoneurons in ALS‐SOD1 mice
A Maatkamp, A Vlug, E Haasdijk… - European Journal of …, 2004 - Wiley Online Library
We have investigated the expression of Hsp25, a heat shock protein constitutively
expressed in motoneurons, in amyotrophic lateral sclerosis (ALS) mice that express G93A …
expressed in motoneurons, in amyotrophic lateral sclerosis (ALS) mice that express G93A …
Caffeic acid and resveratrol ameliorate cellular damage in cell and Drosophila models of spinocerebellar ataxia type 3 through upregulation of Nrf2 pathway
Polyglutamine (polyQ)-expanded mutant ataxin-3 protein, which is prone to misfolding and
aggregation, leads to cerebellar neurotoxicity in spinocerebellar ataxia type 3 (SCA3), an …
aggregation, leads to cerebellar neurotoxicity in spinocerebellar ataxia type 3 (SCA3), an …
Oxidative stress protection by exogenous delivery of rhHsp70 chaperone to the retinal pigment epithelium (RPE), a possible therapeutic strategy against RPE …
A Subrizi, E Toropainen, E Ramsay… - Pharmaceutical …, 2015 - Springer
Purpose To measure the cytoprotective effects of rhHsp70 against oxidative stress and study
its cellular uptake, intracellular and intraocular distribution in the retinal pigment epithelium …
its cellular uptake, intracellular and intraocular distribution in the retinal pigment epithelium …