Yang Song-Yu, He Xue-Ying, Schulz Horst
Department of Neurochemistry, New York State Institute for Basic Research in Developmental Disabilities, Staten Island, 10314, USA.
FEBS J. 2005 Oct;272(19):4874-83. doi: 10.1111/j.1742-4658.2005.04911.x.
3-Hydroxyacyl-CoA dehydrogenase (HAD) functions in mitochondrial fatty acid beta-oxidation by catalyzing the oxidation of straight chain 3-hydroxyacyl-CoAs. HAD has a preference for medium chain substrates, whereas short chain 3-hydroxyacyl-CoA dehydrogenase (SCHAD) acts on a wide spectrum of substrates, including steroids, cholic acids, and fatty acids, with a preference for short chain methyl-branched acyl-CoAs. Therefore, HAD should not be referred to as SCHAD. SCHAD is not a member of the HAD family, but instead, belongs to the short chain dehydrogenase/reductase superfamily. Previously reported cases of SCHAD deficiency are due to an inherited HAD deficiency. SCHAD, also known as 17beta-hydroxysteroid dehydrogenase type 10, is important in brain development and aging. Abnormal levels of SCHAD in certain brain regions may contribute to the pathogenesis of some neural disorders. The human SCHAD gene and its protein product, SCHAD, are potential targets for intervention in conditions, such as Alzheimer's disease, Parkinson's disease, and an X-linked mental retardation, that may arise from the impaired degradation of branched chain fatty acid and isoleucine.
3-羟酰基辅酶A脱氢酶（HAD）通过催化直链3-羟酰基辅酶A的氧化在线粒体脂肪酸β-氧化过程中发挥作用。HAD更倾向于中链底物，而短链3-羟酰基辅酶A脱氢酶（SCHAD）作用于多种底物，包括类固醇、胆酸和脂肪酸，更倾向于短链甲基支链酰基辅酶A。因此，不应将HAD称为SCHAD。SCHAD不是HAD家族的成员，而是属于短链脱氢酶/还原酶超家族。先前报道的SCHAD缺乏症病例是由于遗传性HAD缺乏。SCHAD也被称为10型17β-羟类固醇脱氢酶，在大脑发育和衰老过程中很重要。某些脑区中SCHAD水平异常可能导致一些神经疾病的发病机制。人类SCHAD基因及其蛋白质产物SCHAD是干预某些疾病（如阿尔茨海默病、帕金森病和X连锁智力迟钝）的潜在靶点，这些疾病可能源于支链脂肪酸和异亮氨酸降解受损。
