Jeong Byung-Hoon, Kim Nam-Ho, Kim Jae-Il, Carp Richard I, Kim Yong-Sun
Ilsong Institute of Life Science, Hallym University, Ilsong Building, 1605-4 Gwanyang-dong, Dongan-gu, Anyang, Kyounggi-do, 431-060, South Korea.
New York State Institute for Basic Research in Developmental Disabilities, Staten Island, NY, 10314, USA.
J Hum Genet. 2005;50(6):311-314. doi: 10.1007/s10038-005-0254-8. Epub 2005 Jun 3.
Association between sporadic Creutzfeldt-Jakob disease (CJD) and the prion-like protein gene (PRND) has been reported in the German population. To investigate whether the PRND polymorphisms are associated with an increased risk for developing sporadic CJD in the Korean population, we compared the genotype and allele frequencies of PRND polymorphisms in 110 sporadic CJD patients with those in 102 healthy Koreans. Two polymorphisms (P56L, T174 M) in Koreans were found in the open reading frame (ORF) of PRND. One heterozygote of P56L was observed in normal controls but not in sporadic CJD patients. A strong significant difference of PRND genotype frequency at codon 174 was found between the normal Korean population and various European populations. In contrast to results in the German population, our study did not show a significant difference in PRND genotype or allele frequency at codon 174 between sporadic CJD and normal controls. This was the first genetic association study of the ORF of PRND in an Asian CJD population.
德国人群中已报道散发性克雅氏病(CJD)与朊蛋白样蛋白基因(PRND)之间存在关联。为了研究PRND基因多态性是否与韩国人群散发性CJD发病风险增加相关,我们比较了110例散发性CJD患者与102例健康韩国人的PRND基因多态性的基因型和等位基因频率。在韩国人中发现PRND的开放阅读框(ORF)中有两个多态性(P56L、T174M)。在正常对照中观察到1例P56L杂合子,但散发性CJD患者中未观察到。在韩国正常人群与不同欧洲人群之间,发现第174密码子的PRND基因型频率存在显著差异。与德国人群的结果相反,我们的研究未显示散发性CJD与正常对照之间在第174密码子的PRND基因型或等位基因频率上存在显著差异。这是亚洲CJD人群中首次对PRND的ORF进行的基因关联研究。