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瑞氏综合征中的组织肉碱

Tissue carnitine in Reye syndrome.

作者信息

Willner J H, Chutorian A M, DiMauro S

出版信息

Ann Neurol. 1978 Nov;4(5):468-9. doi: 10.1002/ana.410040514.

DOI:10.1002/ana.410040514
PMID:736529
Abstract

Skeletal muscle carnitine palmityltransferase and muscle or liver carnitine content were determined in biopsies from children during and after attacks of Reye syndrome. No consistent abnormality of enzyme or cofactor was found. Reye syndrome is biochemically distinct from the clinically similar syndrome of systemic carnitine deficiency.

摘要

在患有瑞氏综合征的儿童发作期间及发作后进行活检,测定其骨骼肌肉碱棕榈酰转移酶以及肌肉或肝脏的肉碱含量。未发现酶或辅助因子有一致的异常情况。瑞氏综合征在生化方面与临床上类似的全身性肉碱缺乏综合征不同。

相似文献

1
Tissue carnitine in Reye syndrome.瑞氏综合征中的组织肉碱
Ann Neurol. 1978 Nov;4(5):468-9. doi: 10.1002/ana.410040514.
2
[Alterations of mitochondria in metabolic diseases. Carnitine deficiency, carnitine palmitoyltransferase deficiency and beta oxidation].[代谢性疾病中线粒体的改变。肉碱缺乏、肉碱棕榈酰转移酶缺乏与β氧化]
Acta Neurol (Napoli). 1989 Oct;11(5):330-4.
3
Muscle carnitine palmityltransferase deficiency: a case with enzyme deficiency in cultured fibroblasts.
Ann Neurol. 1978 Nov;4(5):465-7. doi: 10.1002/ana.410040513.
4
Sensitive assay of carnitine palmitoyl transferase activity in tissue homogenates with a modified spectrophotometric method for enzymatic carnitine determination.采用改良分光光度法测定酶促肉碱,对组织匀浆中肉碱棕榈酰转移酶活性进行灵敏检测。
Clin Chim Acta. 1983 Dec 15;135(2):247-51. doi: 10.1016/0009-8981(83)90142-0.
5
Palmitate oxidation in human muscle: comparison to CPT and carnitine.人体肌肉中的棕榈酸氧化:与肉碱棕榈酰转移酶及肉碱的比较
Muscle Nerve. 1982 Mar;5(3):226-31. doi: 10.1002/mus.880050309.
6
Rate-dependent distal renal tubular acidosis and carnitine palmitoyltransferase I deficiency.
Pediatr Res. 1994 Nov;36(5):582-8. doi: 10.1203/00006450-199411000-00007.
7
Measurements of carnitine palmitoyl-transferase I and II.肉碱棕榈酰转移酶I和II的测定
Neurology. 1981 Mar;31(3):361-2. doi: 10.1212/wnl.31.3.361.
8
Beta-oxidation enzymes in normal human muscle and in muscle from a patient with an unusual form of myopathic carnitine deficiency.正常人肌肉以及患有罕见形式肌病性肉碱缺乏症患者肌肉中的β-氧化酶。
Muscle Nerve. 1985 Oct;8(8):672-5. doi: 10.1002/mus.880080809.
9
Recurrent myoglobinuria and muscle carnitine palmityltransferase deficiency.复发性肌红蛋白尿与肌肉肉碱棕榈酰转移酶缺乏症
J Pediatr. 1977 Aug;91(2):247-50. doi: 10.1016/s0022-3476(77)80821-4.
10
[Lipid metabolism disorder in skeletal musculature (carnitine palmitoyltransferase deficiency) and paroxysmal myoglobinuria].
Zh Nevropatol Psikhiatr Im S S Korsakova. 1980;80(11):1623-8.

引用本文的文献

1
Reye syndrome.瑞氏综合征
Neurol Clin. 1985 Feb;3(1):95-115. doi: 10.1016/S0733-8619(18)31058-2.
2
l-Carnitine. A preliminary review of its pharmacokinetics, and its therapeutic use in ischaemic cardiac disease and primary and secondary carnitine deficiencies in relationship to its role in fatty acid metabolism.左旋肉碱。对其药代动力学、在缺血性心脏病中的治疗应用以及与脂肪酸代谢作用相关的原发性和继发性肉碱缺乏症的初步综述。
Drugs. 1987 Jul;34(1):1-24. doi: 10.2165/00003495-198734010-00001.