Kemp Bruce E
St. Vincent's Institute of Medical Research, Fitzroy, Victoria, Australia.
J Clin Invest. 2004 Jan;113(2):182-4. doi: 10.1172/JCI20846.
Conserved pairs of CBS sequence motifs (named after cystathionine beta-synthase) found in a wide variety of proteins associate to form Bateman domains. A new study establishes that Bateman domains bind adenosyl compounds and regulate IMP dehydrogenase, CBS, chloride channels, and AMP-activated protein kinase. This discovery reveals how mutations in CBS sequences in these proteins cause hereditary diseases and provides a rich vista of conceptual opportunities for therapies in energy metabolism, obesity, diabetes, cancer, antivirals, and immunosuppression.
在多种蛋白质中发现的保守的CBS序列基序对(以胱硫醚β-合酶命名)相互关联形成贝特曼结构域。一项新研究证实,贝特曼结构域可结合腺苷化合物,并调节肌苷酸脱氢酶、CBS、氯离子通道和AMP激活的蛋白激酶。这一发现揭示了这些蛋白质中CBS序列的突变如何导致遗传性疾病,并为能量代谢、肥胖、糖尿病、癌症、抗病毒和免疫抑制治疗提供了丰富的概念性机会。