Vulliamy T, Beutler E, Luzzatto L
Department of Haematology, Royal Postgraduate Medical School, Hammersmith Hospital, London, UK.
Hum Mutat. 1993;2(3):159-67. doi: 10.1002/humu.1380020302.
Glucose-6-phosphate dehydrogenase (G6PD) is remarkable for its genetic diversity in humans. Many variants of G6PD have been described with wide ranging levels of enzyme activity and associated clinical symptoms. Fifty-eight different mutations have now been identified and these account for 97 named G6PD variants. The mutations are almost exclusively missense mutations, causing single amino acid substitutions. They are spread throughout the coding region of the gene, although there appears to be a cluster of mutations that cause a more severe clinical phenotype towards the 3' end of the gene. The absence of large deletions, frameshift mutations and nonsense mutations is consistent with the notion that a total lack of G6PD activity would be lethal.
葡萄糖-6-磷酸脱氢酶(G6PD)在人类中因其遗传多样性而引人注目。已描述了许多G6PD变体,其酶活性水平和相关临床症状差异很大。现已鉴定出58种不同的突变,这些突变构成了97种命名的G6PD变体。这些突变几乎都是错义突变,导致单个氨基酸替换。它们分布在基因的整个编码区域,尽管似乎有一组突变在基因的3'端导致更严重的临床表型。不存在大的缺失、移码突变和无义突变与G6PD活性完全缺乏将是致命的这一观点一致。