Journal
NEUROBIOLOGY OF AGING
Volume 31, Issue 6, Pages 969-985Publisher
ELSEVIER SCIENCE INC
DOI: 10.1016/j.neurobiolaging.2008.07.005
Keywords
Amyotrophic lateral sclerosis; Motor neurones; VAPB; Heat shock proteins; HSPB1; HSPB8
Categories
Funding
- Motor Neurone Disease Association
- The Henry Smith Charity
- American ALS Association
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The aim of this study was to quantify spinal cord expression of genes known to cause familial amyotrophic lateral sclerosis (FALS) or influence survival in a large cohort of sporadic cases of ALS (SALS), in order to determine their relevance to pathogenic mechanisms occurring in SALS. The expression of superoxide dismutase 1 (SOD1), vesicle associated membrane protein (VAPB), senataxin (SETX), dynactin (DCTN1), vascular endothelial growth factor (VEGF), insulin-like growth factor-1 (IGF1), the small heat shock proteins, HSPB1 and HSPB8, and three genes activated during disease progression, caspases-1 and -3 and glial fibrillary acidic protein (GFAP), were quantified. Robust changes in the expression of four genes were found, VAPB mRNA levels were decreased in the spinal cord of ALS patients compared to controls (p < 0.006), whilst HSPB1, HSPB8 and caspase-1 showed significant increases (1.5-2.3-fold). Expression of VAPB mRNA and protein was predominantly localised to large motor neurones further supporting the relevance of this finding to disease progression occurring in SALS. (C) 2008 Elsevier Inc. All rights reserved.
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