齐-韦二氏综合征中的胆固醇生物合成：患者成纤维细胞中甲羟戊酸激酶、甲羟戊酸-5'-焦磷酸脱羧酶和异戊烯焦磷酸异构酶活性正常，但肝脏中甲羟戊酸激酶活性不足。 - Suppr

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超能文献

Cholesterol biosynthesis in Zellweger syndrome: normal activity of mevalonate kinase, mevalonate-5'-pyrophosphate decarboxylase and IPP-isomerase in patients' fibroblasts but deficient mevalonate kinase activity in liver.

作者信息

Wanders R J, Romeijn G J

机构信息

Department of Clinical Chemistry, University Hospital Amsterdam, The Netherlands.

出版信息

J Inherit Metab Dis. 1996;19(2):193-6. doi: 10.1007/BF01799427.

DOI:10.1007/BF01799427

PMID:8739963

Abstract

摘要

相似文献

Cholesterol biosynthesis in Zellweger syndrome: normal activity of mevalonate kinase, mevalonate-5'-pyrophosphate decarboxylase and IPP-isomerase in patients' fibroblasts but deficient mevalonate kinase activity in liver.齐-韦二氏综合征中的胆固醇生物合成：患者成纤维细胞中甲羟戊酸激酶、甲羟戊酸-5'-焦磷酸脱羧酶和异戊烯焦磷酸异构酶活性正常，但肝脏中甲羟戊酸激酶活性不足。

J Inherit Metab Dis. 1996;19(2):193-6. doi: 10.1007/BF01799427.

Cholesterol biosynthesis, peroxisomes and peroxisomal disorders: mevalonate kinase is not only deficient in Zellweger syndrome but also in rhizomelic chondrodysplasia punctata.胆固醇生物合成、过氧化物酶体与过氧化物酶体疾病：甲羟戊酸激酶不仅在泽尔韦格综合征中缺乏，在点状软骨发育不良中也缺乏。

J Inherit Metab Dis. 1998 Jun;21(3):309-12. doi: 10.1023/a:1005353129761.

Cholesterol biosynthesis is not defective in peroxisome biogenesis defective fibroblasts.在过氧化物酶体生物发生缺陷的成纤维细胞中，胆固醇生物合成并无缺陷。

Mol Genet Metab. 2003 Nov;80(3):290-5. doi: 10.1016/S1096-7192(03)00143-4.

Compartmentalization of cholesterol biosynthesis. Conversion of mevalonate to farnesyl diphosphate occurs in the peroxisomes.胆固醇生物合成的区室化。甲羟戊酸转化为法尼基二磷酸发生在过氧化物酶体中。

J Biol Chem. 1996 Jan 19;271(3):1784-8. doi: 10.1074/jbc.271.3.1784.

Differential deficiency of mevalonate kinase and phosphomevalonate kinase in patients with distinct defects in peroxisome biogenesis: evidence for a major role of peroxisomes in cholesterol biosynthesis.过氧化物酶体生物发生存在不同缺陷的患者中，甲羟戊酸激酶和磷酸甲羟戊酸激酶的差异性缺乏：过氧化物酶体在胆固醇生物合成中起主要作用的证据

Biochem Biophys Res Commun. 1998 Jun 29;247(3):663-7. doi: 10.1006/bbrc.1998.8836.

Absence of functional peroxisomes does not lead to deficiency of enzymes involved in cholesterol biosynthesis.功能性过氧化物酶体的缺失不会导致参与胆固醇生物合成的酶缺乏。

J Lipid Res. 2002 Jan;43(1):90-8.

Effects of different nutritional conditions on chick liver mevalonate-activating enzymes.不同营养条件对雏鸡肝脏甲羟戊酸激活酶的影响。

Biochim Biophys Acta. 1986 Feb 28;875(3):605-9. doi: 10.1016/0005-2760(86)90083-4.

Control of phosphorylation and decarboxylation of mevalonic acid and its metabolites in cultured human fibroblasts and in rat liver in vivo.体内培养的人成纤维细胞和大鼠肝脏中甲羟戊酸及其代谢产物的磷酸化和脱羧作用的调控

J Biol Chem. 1981 Jun 25;256(12):6170-3.

Changes in chick liver and brain mevalonate kinase, mevalonate-5-phosphate kinase and mevalonate-5-pyrophosphate decarboxylase during development.雏鸡肝脏和大脑中羟甲基戊二酸单酰辅酶A还原酶、磷酸甲羟戊酸激酶和甲羟戊酸-5-焦磷酸脱羧酶在发育过程中的变化。

Int J Biochem. 1984;16(7):845-7. doi: 10.1016/0020-711x(84)90200-3.

Studies on the diurnal rhythm of mevalonate metabolism by sterol and nonsterol pathways and of mevalonate-activating enzymes.

Int J Biochem. 1985;17(2):275-8. doi: 10.1016/0020-711x(85)90127-2.

引用本文的文献

Disturbed cholesterol homeostasis in a peroxisome-deficient PEX2 knockout mouse model.过氧化物酶体缺陷型PEX2基因敲除小鼠模型中胆固醇稳态紊乱

Mol Cell Biol. 2004 Jan;24(1):1-13. doi: 10.1128/MCB.24.1.1-13.2004.

J Inherit Metab Dis. 1998 Jun;21(3):309-12. doi: 10.1023/a:1005353129761.

本文引用的文献

Farnesyl-diphosphate synthase is localized in peroxisomes.法尼基二磷酸合酶定位于过氧化物酶体中。

J Biol Chem. 1994 May 13;269(19):14165-9.

Differential protein import deficiencies in human peroxisome assembly disorders.人类过氧化物酶体组装障碍中蛋白质导入的差异缺陷

J Cell Biol. 1994 May;125(4):755-67. doi: 10.1083/jcb.125.4.755.

Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome biogenesis disorders.过氧化物酶体生物合成障碍互补组2由过氧化物酶体靶向信号1（PTS1）受体基因PXR1的突变所定义。

Nat Genet. 1995 Feb;9(2):115-25. doi: 10.1038/ng0295-115.

Cholesterol biosynthesis in dermal fibroblasts from patients with metabolic disorders of peroxisomal origin.过氧化物酶体起源的代谢紊乱患者皮肤成纤维细胞中的胆固醇生物合成。

Eur J Clin Invest. 1995 Jan;25(1):59-67. doi: 10.1111/j.1365-2362.1995.tb01527.x.

Reduced cellular cholesterol content in peroxisome-deficient fibroblasts is associated with impaired uptake of the patient's low density lipoprotein and with reduced cholesterol synthesis.过氧化物酶体缺陷的成纤维细胞中细胞胆固醇含量降低，这与患者低密度脂蛋白摄取受损以及胆固醇合成减少有关。

J Lipid Res. 1995 Jun;36(6):1385-91.

Identification of three distinct peroxisomal protein import defects in patients with peroxisome biogenesis disorders.在过氧化物酶体生物发生障碍患者中鉴定出三种不同的过氧化物酶体蛋白导入缺陷。

J Cell Sci. 1995 May;108 ( Pt 5):1817-29. doi: 10.1242/jcs.108.5.1817.

J Biol Chem. 1981 Jun 25;256(12):6170-3.

3-Hydroxy-3-methylglutaryl-coenzyme A reductase is present in peroxisomes in normal rat liver cells.3-羟基-3-甲基戊二酰辅酶A还原酶存在于正常大鼠肝细胞的过氧化物酶体中。

Proc Natl Acad Sci U S A. 1985 Feb;82(3):770-4. doi: 10.1073/pnas.82.3.770.

Infantile Refsum disease: an inherited peroxisomal disorder. Comparison with Zellweger syndrome and neonatal adrenoleukodystrophy.婴儿型雷夫叙姆病：一种遗传性过氧化物酶体病。与泽尔韦格综合征和新生儿肾上腺脑白质营养不良的比较。

Eur J Pediatr. 1987 Sep;146(5):477-83. doi: 10.1007/BF00441598.

Presence of individual enzymes of cholesterol biosynthesis in rat liver peroxisomes.

Arch Biochem Biophys. 1990 Nov 1;282(2):318-25. doi: 10.1016/0003-9861(90)90123-g.

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