一例肌肉肉碱棕榈酰转移酶缺乏症患者对中链甘油三酯和长链甘油三酯的不同生酮反应。 - Suppr

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超能文献

Different ketogenic response to medium-chain triglycerides and to long-chain triglycerides in a case of muscular carnitine palmitoyltransferase deficiency.

作者信息

Sugiyama N, Wada Y, Morishita H, Nonaka I

出版信息

J Inherit Metab Dis. 1982;5(4):233-4. doi: 10.1007/BF02179151.

DOI:10.1007/BF02179151

PMID:6820447

Abstract

摘要

相似文献

Different ketogenic response to medium-chain triglycerides and to long-chain triglycerides in a case of muscular carnitine palmitoyltransferase deficiency.一例肌肉肉碱棕榈酰转移酶缺乏症患者对中链甘油三酯和长链甘油三酯的不同生酮反应。

J Inherit Metab Dis. 1982;5(4):233-4. doi: 10.1007/BF02179151.

Heterogeneity of carnitine-palmitoyltransferase deficiency.肉碱-棕榈酰转移酶缺乏症的异质性。

J Neurol Sci. 1981 May;50(2):207-15. doi: 10.1016/0022-510x(81)90167-2.

Carnitine palmityltransferase deficiency and fixed muscle weakness.肉碱棕榈酰转移酶缺乏症与固定性肌肉无力。

Arch Neurol. 1990 Jan;47(1):13. doi: 10.1001/archneur.1990.00530010019010.

Fasting hypoglycemia resulting from hepatic carnitine palmitoyl transferase deficiency.因肝脏肉碱棕榈酰转移酶缺乏导致的空腹低血糖症。

J Pediatr. 1981 May;98(5):742-6. doi: 10.1016/s0022-3476(81)80834-7.

Carnitine palmitoyl transferase deficiency with an atypical presentation and ultrastructural mitochondrial abnormalities.肉碱棕榈酰转移酶缺乏症伴非典型表现及超微结构线粒体异常。

J Neurol Neurosurg Psychiatry. 1987 Aug;50(8):1060-2. doi: 10.1136/jnnp.50.8.1060.

[Lipid storage myopathies--carnitine deficiency, carnitine palmitoyltransferase deficiency, pyruvate decarboxylase deficiency].[脂质贮积性肌病——肉碱缺乏症、肉碱棕榈酰转移酶缺乏症、丙酮酸脱羧酶缺乏症]

Nihon Rinsho. 1990 Jul;48(7):1510-6.

[Heterogeneity of carnitine palmitoyltransferase deficiencies. Deficiency of CPT I in the hepatic form and CPT II in the muscular form].

Arch Fr Pediatr. 1985 Aug-Sep;42 Suppl 1:613-7.

Deficiency of carnitine palmitoyltransferase I.

J Child Neurol. 1989 Jul;4(3):198-203. doi: 10.1177/088307388900400310.

[Intolerance to exercise caused by carnitine palmitoyltransferase deficiency].

Rev Neurol (Paris). 1990;146(3):231-4.

[Clinical and biochemical approach to mitochondrial cytopathy--carnitine palmitoyltransferase deficiency].线粒体细胞病——肉碱棕榈酰转移酶缺乏症的临床与生化研究方法

No To Hattatsu. 1987 Mar;19(2):140-9.

引用本文的文献

Palm Oil in Lipid-Based Formulations and Drug Delivery Systems.棕榈油在脂质体制剂和药物传递系统中的应用。

Biomolecules. 2019 Feb 13;9(2):64. doi: 10.3390/biom9020064.

Biochemical evidence of carnitine effect on propionate elimination.

J Inherit Metab Dis. 1984;7(3):137-8. doi: 10.1007/BF01801775.

J Neurol Neurosurg Psychiatry. 1987 Aug;50(8):1060-2. doi: 10.1136/jnnp.50.8.1060.

Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes syndrome and NADH-CoQ reductase deficiency.线粒体肌病、脑病、乳酸酸中毒和卒中样发作综合征以及NADH-辅酶Q还原酶缺乏症。

J Inherit Metab Dis. 1986;9(3):301-4. doi: 10.1007/BF01799670.

Multiple enzyme defects in mitochondria of a case with congenital lactic acidosis and hyperammonaemia.

J Inherit Metab Dis. 1992;15(5):809-14. doi: 10.1007/BF01800026.

本文引用的文献

PALMITYL-COA:CARNITINE PALMITYLTRANSFERASE. PURIFICATION FROM CALF-LIVER MITOCHONDRIA AND SOME PROPERTIES OF THE ENZYME.棕榈酰辅酶A：肉碱棕榈酰转移酶。从小牛肝脏线粒体中纯化及该酶的一些性质

Biochim Biophys Acta. 1964 Jul 8;89:95-108.

An improved and simplified radioisotopic assay for the determination of free and esterified carnitine.一种用于测定游离和酯化肉碱的改进型简化放射性同位素测定法。

J Lipid Res. 1976 May;17(3):277-81.

Microdetermination of (-)carnitine and carnitine acetyltransferase activity.（-）肉碱及肉碱乙酰转移酶活性的微量测定

Anal Biochem. 1977 May 1;79(1-2):190-201. doi: 10.1016/0003-2697(77)90393-1.

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