中链酰基辅酶A脱氢酶缺乏症：患者和成纤维细胞系中，成纤维细胞酰基辅酶A脱氢酶对辛酰辅酶A的表观米氏常数（Km）和最大反应速度（Vmax）值。 - Suppr | 超能文献

Medium chain acyl-CoA dehydrogenase deficiency: apparent Km and Vmax values for fibroblast acyl-CoA dehydrogenase towards octanoyl CoA in patient and control cell lines.

作者信息

Gregersen N, Kølvraa S

出版信息

J Inherit Metab Dis. 1984;7 Suppl 2:105-6. doi: 10.1007/978-94-009-5612-4_27.

DOI:10.1007/978-94-009-5612-4_27

Abstract

摘要

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Medium chain acyl-CoA dehydrogenase deficiency: apparent Km and Vmax values for fibroblast acyl-CoA dehydrogenase towards octanoyl CoA in patient and control cell lines.中链酰基辅酶A脱氢酶缺乏症：患者和成纤维细胞系中，成纤维细胞酰基辅酶A脱氢酶对辛酰辅酶A的表观米氏常数（Km）和最大反应速度（Vmax）值。

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2

Catalytic defect of medium-chain acyl-coenzyme A dehydrogenase deficiency. Lack of both cofactor responsiveness and biochemical heterogeneity in eight patients.中链酰基辅酶A脱氢酶缺乏症的催化缺陷。八例患者缺乏辅因子反应性和生化异质性。

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Interaction of acyl coenzyme A substrates and analogues with pig kidney medium-chain acyl-coA dehydrogenase.酰基辅酶A底物及类似物与猪肾中链酰基辅酶A脱氢酶的相互作用

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Genetic deficiency of short-chain acyl-coenzyme A dehydrogenase in cultured fibroblasts from a patient with muscle carnitine deficiency and severe skeletal muscle weakness.一名患有肌肉肉碱缺乏症和严重骨骼肌无力的患者的培养成纤维细胞中短链酰基辅酶A脱氢酶的基因缺陷。

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Facile and restricted pathways for the dissociation of octenoyl-CoA from the medium-chain fatty acyl-CoA dehydrogenase (MCAD)-FADH2-octenoyl-CoA charge-transfer complex: energetics and mechanism of suppression of the enzyme's oxidase activity.辛烯酰辅酶A从中链脂肪酰辅酶A脱氢酶（MCAD）-FADH2-辛烯酰辅酶A电荷转移复合物解离的简易途径和受限途径：抑制该酶氧化酶活性的能量学和机制

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Heptanoic and medium branched-chain fatty acids as anaplerotic treatment for medium chain acyl-CoA dehydrogenase deficiency.庚酸和中链支链脂肪酸作为中链酰基辅酶 A 脱氢酶缺乏症的补充治疗。

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2

Catalytic defect of medium-chain acyl-coenzyme A dehydrogenase deficiency. Lack of both cofactor responsiveness and biochemical heterogeneity in eight patients.中链酰基辅酶A脱氢酶缺乏症的催化缺陷。八例患者缺乏辅因子反应性和生化异质性。

J Clin Invest. 1985 Sep;76(3):963-9. doi: 10.1172/JCI112096.