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Poloxamer 188 decreases membrane toxicity of mutant SOD1 and ameliorates pathology observed in SOD1 mouse model for ALS.

Abstract：

:Here we report a gain in function for mutant (mt) superoxide dismutase I (SOD1), a cause of familial amyotrophic lateral sclerosis (FALS), wherein small soluble oligomers of mtSOD1 acquire a membrane toxicity. Phosphatidylglycerol (PG) lipid domains are selectively targeted, which could result in membrane damage or "toxic channels" becoming active in the bilayer. This PG-selective SOD1-mediated membrane toxicity is largely reversible in vitro by a widely-available FDA-approved surfactant and membrane-stabilizer P188. Treatment of G93ASOD1 transgenic mice with P188 significantly delayed symptoms onset, extended survival and decreased motoneuron death. The use of P188 or an analogue, which targets mtSOD1 misfolding-induced membrane toxicity, may provide a new direction for ALS treatment.

journal_name

Neurobiol Dis

journal_title

Neurobiology of disease

authors

Riehm JJ,Wang L,Ghadge G,Teng M,Correa AM,Marks JD,Roos RP,Allen MJ

doi

10.1016/j.nbd.2018.03.014

subject

Has Abstract

pub_date

2018-07-01 00:00:00

pages

115-126

eissn

0969-9961

issn

1095-953X

pii

S0969-9961(18)30093-7

journal_volume

115

pub_type

杂志文章

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