Juvenile-onset glycogen storage disease type II with novel mutations in acid alpha-glucosidase gene.

Lam, C W; Yuen, Y P; Chan, K Y; Tong, S F; Lai, C K; Chow, T C; Lee, K C; Chan, Y W et al. · Neurology · 2003

case_report · Level V

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Abstract

The authors describe two novel mutations of the acid alpha-glucosidase gene, P361L and R437C, which define the juvenile-onset glycogen storage disease type II (GSDII) in a 16-year-old Chinese patient. The asymptomatic 13-year-old brother of the proband is also a compound heterozygote of the two mutations. These results confirm that intrafamilial phenotypic variation of juvenile-onset GSDII is ethnically diverse and suggest the contribution of other genes to the phenotypic variability of GSDII.

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