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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.

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
PMID:6434856
Abstract
摘要

相似文献

1
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。
J Inherit Metab Dis. 1984;7 Suppl 2:105-6. doi: 10.1007/978-94-009-5612-4_27.
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.
3
Measurement of short-chain acyl-CoA dehydrogenase (SCAD) in cultured skin fibroblasts with hexanoyl-CoA as a competitive inhibitor to eliminate the contribution of medium-chain acyl-CoA dehydrogenase.以己酰辅酶A作为竞争性抑制剂,在培养的皮肤成纤维细胞中测量短链酰基辅酶A脱氢酶(SCAD),以消除中链酰基辅酶A脱氢酶的影响。
Clin Chim Acta. 1994 Sep;229(1-2):99-106. doi: 10.1016/0009-8981(94)90232-1.
4
An acyl-coenzyme A dehydrogenase assay utilizing the ferricenium ion.一种利用铁离子的酰基辅酶A脱氢酶测定法。
Anal Biochem. 1990 May 1;186(2):280-4. doi: 10.1016/0003-2697(90)90080-s.
5
Stereoselective interaction of 2-halo-acyl-CoA derivatives with medium chain acyl-CoA dehydrogenase from pig kidney.2-卤代酰基辅酶A衍生物与猪肾中链酰基辅酶A脱氢酶的立体选择性相互作用。
Arch Biochem Biophys. 1993 Apr;302(1):85-91. doi: 10.1006/abbi.1993.1184.
6
Reductive half-reaction of medium-chain fatty acyl-CoA dehydrogenase utilizing octanoyl-CoA/octenoyl-CoA as a physiological substrate/product pair: similarity in the microscopic pathways of octanoyl-CoA oxidation and octenoyl-CoA binding.利用辛酰辅酶A/辛烯酰辅酶A作为生理底物/产物对的中链脂肪酰辅酶A脱氢酶的还原半反应:辛酰辅酶A氧化和辛烯酰辅酶A结合微观途径的相似性
Biochemistry. 1994 Jul 26;33(29):8833-41. doi: 10.1021/bi00195a027.
7
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
Biochemistry. 1987 Jun 16;26(12):3704-10. doi: 10.1021/bi00386a066.
8
Measurement of acyl-CoA dehydrogenase activity in cultured skin fibroblasts and blood platelets.培养的皮肤成纤维细胞和血小板中酰基辅酶A脱氢酶活性的测定。
J Inherit Metab Dis. 1992;15(5):727-32. doi: 10.1007/BF01800014.
9
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。
J Clin Invest. 1988 Jan;81(1):171-5. doi: 10.1172/JCI113290.
10
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
Biochemistry. 1995 Jul 25;34(29):9434-43. doi: 10.1021/bi00029a019.

引用本文的文献

1
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。
Mol Genet Metab. 2023 Nov;140(3):107689. doi: 10.1016/j.ymgme.2023.107689. Epub 2023 Aug 25.
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.