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25-Hydroxycholesterol is involved in the pathogenesis of amyotrophic lateral sclerosis

Title
25-Hydroxycholesterol is involved in the pathogenesis of amyotrophic lateral sclerosis
Author
노민영
Keywords
amyotrophic lateral sclerosis; cholesterol; hydroxycholesterol; 25-hydroxycholesterol; liver X receptor
Issue Date
2017-01
Publisher
IMPACT JOURNALS LLC
Citation
ONCOTARGET, v. 8, no. 7, page. 11855-11867
Abstract
This study aimed to evaluate the levels of three major hydroxycholesterols (24-, 25-, and 27-hydroxycholesterols) in the serum and cerebrospinal fluid (CSF) of patients with amyotrophic lateral sclerosis (ALS), as well as to show their role in the pathogenesis of ALS experimental models. The level of 25-hydroxycholesterol were higher in untreated ALS patients (n = 30) than in controls without ALS (n = 33) and ALS patients treated with riluzole (n = 9) both in their serum and CSF. The level of 25-hydroxycholesterol in the serum of ALS patients were significantly associated with their disease severity and rate of progression. In the motor neuron-like cell line (NSC34) with the human mutant G93A superoxide dismutase 1 gene (mSOD1G93A), 25-hydroxycholesterol induced motor neuronal death/apoptosis via glycogen synthase kinase-3 beta and liver X receptor pathways; riluzole treatment attenuated these effects. The expressions of enzymes that synthesize 25-hydroxycholesterol were significantly increased in the brains of early symptomatic mSOD1G93A mice. Our data, obtained from patients with ALS, a cellular model of ALS, and an animal model of ALS, suggests that 25-hydroxycholesterol could be actively involved in the pathogenesis of ALS, mostly in the early symptomatic disease stage, by mediating neuronal apoptosis.
URI
http://www.oncotarget.com/index.php?journal=oncotarget&page=article&op=view&path[]=14416https://repository.hanyang.ac.kr/handle/20.500.11754/112334
ISSN
1949-2553
DOI
10.18632/oncotarget.14416
Appears in Collections:
RESEARCH INSTITUTE[S](부설연구소) > ETC
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