Linkage of Niemann-Pick disease type C to human chromosome 18.

Carstea, E D; Polymeropoulos, M H; Parker, C C; Detera-Wadleigh, S D; O'Neill, R R; Patterson, M C; Goldin, E; Xiao, H et al. · Proc Natl Acad Sci U S A · 1993

basic_science · Level V

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Abstract

We analyzed the involvement of chromosome 18 in Niemann-Pick disease type C (NPC), an autosomal recessive cholesterol-processing disorder. Within affected offspring, the chromosome 18 parental contributions were identified by using allele-specific microsatellite markers. Significant linkage of NPC to an 18p genomic marker, D18S40, was indicated by a two-point lod score of 3.84. Analysis of meiotic chromosomal breakpoint patterns among the affected individuals indicated that the NPC gene is pericentromerically localized on human chromosome 18.

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