Vesicle associated membrane protein B (VAPB) is decreased in ALS spinal cord

被引：108
作者：
Anagnostou, Georgia [1 ]
Akbar, Mohammed T. [1 ]
Paul, Praveen [1 ]
Angelinetta, Claire [1 ]
Steiner, Timothy J. [1 ]
de Belleroche, Jacqueline [1 ]
机构：
[1] Univ London Imperial Coll Sci Technol & Med, Neurogenet Grp, Dept Cellular & Mol Neurosci, Div Neurosci & Mental Hlth,Fac Med, London W12 0NN, England
来源：
NEUROBIOLOGY OF AGING | 2010年 / 31卷 / 06期
关键词：
Amyotrophic lateral sclerosis; Motor neurones; VAPB; Heat shock proteins; HSPB1; HSPB8; AMYOTROPHIC-LATERAL-SCLEROSIS; HEAT-SHOCK-PROTEIN; ENDOTHELIAL GROWTH-FACTOR; TRANSGENIC MOUSE MODEL; MOTOR-NEURON DEGENERATION; MARIE-TOOTH-DISEASE; PROGRAMMED CELL-DEATH; REAL-TIME PCR; UP-REGULATION; FRONTOTEMPORAL DEMENTIA;
D O I：
10.1016/j.neurobiolaging.2008.07.005
中图分类号：
R592 [老年病学]; C [社会科学总论];
学科分类号：
03 ; 0303 ; 100203 ;
摘要：
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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页码：969 / 985
页数：17
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