Nyhan W L
Department of Pediatrics, University of California, San Diego, La Jolla, California 92093, USA.
Nucleosides Nucleotides Nucleic Acids. 2008 Jun;27(6):559-63. doi: 10.1080/15257770802135745.
Lesch-Nyhan disease is the most severe or complete phenotype of deficiency in hypoxanthineguanine phosphoribosyltransferase; other variant enzymes are found in patients without abnormality in behavior or mental development, and there are intermediate phenotypes in which enzyme activity is intermediate. A considerable number and variety of mutations in the HPRT gene have been discovered.
莱施-奈恩综合征是次黄嘌呤鸟嘌呤磷酸核糖转移酶缺乏最严重或最完全的表型;在行为或智力发育无异常的患者中发现了其他变异酶,还有一些中间表型,其酶活性处于中间水平。已发现HPRT基因中有相当数量和种类的突变。