MST1 functions as a key modulator of neurodegeneration in a mouse model of ALS

Title
MST1 functions as a key modulator of neurodegeneration in a mouse model of ALS
Authors
류훈이재근신진희황상질곽병주Ann C. Mckee이정희Neil W. Kowall임대식최의주
Issue Date
2013-07
Publisher
Proceedings of the National Academy of Sciences of the United States of America
Citation
VOL 110, NO 29-12071
Abstract
Amyotrophic lateral sclerosis (ALS) is an adult-onset neurodegenerative disorder characterized by loss of motor neurons. Dominant mutations in the gene for superoxide dismutase 1 (SOD1) give rise to familial ALS by an unknown mechanism. Here we show that genetic deficiency of mammalian sterile 20-like kinase 1 (MST1) delays disease onset and extends survival in mice expressing the ALS-associated G93A mutant of human SOD1. SOD1(G93A) induces dissociation of MST1 from a redox protein thioredoxin-1 and promotes MST1 activation in spinal cord neurons in a reactive oxygen species– dependent manner. Moreover, MST1 was found to mediate SOD1(G93A)-induced activation of p38 mitogen-activated protein kinase and caspases as well as impairment of autophagy in spinal cord motoneurons of SOD1(G93A) mice. Our findings implicate MST1 as a key determinant of neurodegeneration in ALS.
URI
http://pubs.kist.re.kr/handle/123456789/64626
ISSN
0027-8424
Appears in Collections:
KIST Publication > Article
Files in This Item:
There are no files associated with this item.
Export
RIS (EndNote)
XLS (Excel)
XML


qrcode

Items in DSpace are protected by copyright, with all rights reserved, unless otherwise indicated.

BROWSE