Deleterious variants of FIG4, a phosphoinositide phosphatase, in patients with ALS.

Chow, Clement Y; Landers, John E; Bergren, Sarah K; Sapp, Peter C; Grant, Adrienne E; Jones, Julie M; Everett, Lesley; Lenk, Guy M et al. · Am J Hum Genet · 2009

case_control · Level III

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Abstract

Mutations of the lipid phosphatase FIG4 that regulates PI(3,5)P(2) are responsible for the recessive peripheral-nerve disorder CMT4J. We now describe nonsynonymous variants of FIG4 in 2% (9/473) of patients with amyotrophic lateral sclerosis (ALS) and primary lateral sclerosis (PLS). Heterozygosity for a deleterious allele of FIG4 appears to be a risk factor for ALS and PLS, extending the list of known ALS genes and increasing the clinical spectrum of FIG4-related diseases.

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