NDLI: Levels of DNAJB family members (HSP40) correlate with disease onset in patients with spinocerebellar ataxia type 3

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Levels of DNAJB family members (HSP40) correlate with disease onset in patients with spinocerebellar ataxia type 3

Content Provider	World Health Organization (WHO)-Global Index Medicus
Author	Zijlstra, M. P. Rujano, M. A. Van Waarde, M. A. Vis, E. Brunt, E. R. Kampinga, H. H.
Description	Country affiliation: Netherlands Author Affiliation: Zijlstra MP ( Department of Cell Biology, Section of Radiation and Stress Cell Biology, University Medical Center Groningen, University of Groningen, Ant. Deusinglaan 1, Groningen, The Netherlands.)
Abstract	In polyglutamine disorders, the length of the expanded CAG repeat shows a strong inverse correlation with the age at disease onset, yet up to 50% of the variation in age of onset is determined by other additional factors. Here, we investigated whether variations in the expression of heat shock proteins (HSP) are related to differences in the age of onset in patients with spinocerebellar ataxia (SCA)3. Hereto, we analysed the protein expression levels of HSPA1A (HSP70), HSPA8 (HSC70), DNAJB (HSP40) and HSPB1 (HSP27) in fibroblasts from patients and healthy controls. HSPB1 levels were significantly upregulated in fibroblasts from patients with SCA3, but without relation to age of onset. Exclusively for expression of DNAJB family members, a correlation was found with the age of onset independent of the length of the CAG repeat expansion. This indicates that DNAJB members might be contributors to the variation in age of onset and underlines the possible use of DNAJB proteins as therapeutic targets.
File Format	HTM / HTML
ISSN	0953816X
Issue Number	5
Volume Number	32
e-ISSN	14609568
Journal	European Journal of Neuroscience
Language	English
Publisher	Wiley-Blackwell
Publisher Date	2010-09-01
Publisher Place	France
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Discipline Neuroscience Hsp40 Heat-shock Proteins Biosynthesis Heat-shock Proteins Machado-joseph Disease Genetics Adult Age Of Onset Aged Ataxin-3 Cell Culture Techniques Cell Line Cell Line, Transformed Female Fibroblasts Metabolism Humans Male Middle Aged Nerve Tissue Proteins Nuclear Proteins Repressor Proteins Trinucleotide Repeat Expansion Up-regulation Journal Article Research Support, Non-u.s. Gov't
Content Type	Text
Resource Type	Article
Subject	Neuroscience

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Levels of DNAJB family members (HSP40) correlate with disease onset in patients with spinocerebellar ataxia type 3

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Levels of DNAJB family members (HSP40) correlate with disease onset in patients with spinocerebellar ataxia type 3