短链酰基辅酶A脱氢酶(short-chain acyl-CoA dehydrogenase,SCAD)抑制胰岛素的分泌和糖处理功能,转氨基反应(transamination reaction)障碍引起短链酰基肉碱(short-chain acylcarnit
2在饥饿情况下,中链酰基辅酶A脱氢酶(medium-chain acyl-CoA dehydrogenase,MCAD)缺陷使机体不能产生足够的酮体,出现血浆脂肪酸增高和低血糖症。线粒体内中链(C8-~C12-)辅酶A的中间产
3长链L-3-羟酰基辅酶A脱氢酶 (long-chain L-3-hydroxyacyl-CoA dehydrogenase,LCHAD)缺陷症属常染色体隐性遗传性疾病。发病年龄多在3岁以内。主要表现为饥饿性非酮症性低血糖症
4长链酰基辅酶A脱氢酶(LCHAD)缺陷症主要包括长链酰基辅酶A脱氢酶缺陷症、长链L-3-羟酰基辅酶A脱氢酶(long-chain L-3-hydroxyacyl-CoA dehydrogenase,LCHAD)缺陷症、中链酰基辅酶A
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