Doering C B, Coursey C, Spangler W, Danner D J
Emory University School of Medicine, Department of Genetics, Atlanta, GA 30322, USA.
Gene. 1998 Jun 8;212(2):213-9. doi: 10.1016/s0378-1119(98)00182-6.
These studies were designed to demonstrate the structural and functional similarity of murine branched chain alpha-ketoacid dehydrogenase and its regulation by the complex-specific kinase. Nucleotide sequence and deduced amino acid sequence for the kinase cDNA demonstrate a highly conserved coding sequence between mouse and human. Tissue-specific expression in adult mice parallels that reported in other mammals. Kinase expression in female liver is influenced by circadian rhythm. Of special interest is the fluctuating expression of this kinase during embryonic development against the continuing increase in the catalytic subunits of this mitochondrial complex during development. The need for regulation of the branched chain alpha-ketoacid dehydrogenase complex by kinase expression during embryogenesis is not understood. However, the similarity of murine branched chain alpha-ketoacid dehydrogenase and its kinase to the human enzyme supports the use of this animal as a model for the human system.
这些研究旨在证明小鼠支链α-酮酸脱氢酶的结构和功能相似性及其受复合物特异性激酶的调节。激酶cDNA的核苷酸序列和推导的氨基酸序列表明，小鼠和人类之间的编码序列高度保守。成年小鼠中的组织特异性表达与其他哺乳动物中报道的情况相似。雌性肝脏中的激酶表达受昼夜节律影响。特别令人感兴趣的是，在胚胎发育过程中，这种激酶的表达波动，而在此期间该线粒体复合物的催化亚基持续增加。目前尚不清楚胚胎发生过程中通过激酶表达来调节支链α-酮酸脱氢酶复合物的必要性。然而，小鼠支链α-酮酸脱氢酶及其激酶与人类酶的相似性支持将这种动物用作人类系统的模型。
