Two novel mutations of the glycine receptor gene in a Taiwanese hyperekplexia family.

Tsai, C H; Chang, F C; Su, Y C; Tsai, F J; Lu, M K; Lee, C C; Kuo, C C; Yang, Y W et al. · Neurology · 2004

case_report · Level V

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Abstract

The authors report a Taiwanese family with autosomal recessive hyperekplexia. Two novel mutations, W96C (from the paternal allele) and R344X (from the maternal allele), which are located in exon 4 and exon 7 of the GLRA1 gene, were identified in this family. A series of electrophysiologic investigations were conducted in one of the probands, and the results suggest that the "startle center" is located subcortically.

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