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Role of lysosomal acid ceramidase in the metabolism of ceramide in human skin fibroblasts.

作者信息

Chen W W, Moser A B, Moser H W

出版信息

Arch Biochem Biophys. 1981 May;208(2):444-55. doi: 10.1016/0003-9861(81)90531-2.

DOI:10.1016/0003-9861(81)90531-2
PMID:7259198
Abstract
摘要

相似文献

1
Role of lysosomal acid ceramidase in the metabolism of ceramide in human skin fibroblasts.溶酶体酸性神经酰胺酶在人皮肤成纤维细胞中神经酰胺代谢中的作用
Arch Biochem Biophys. 1981 May;208(2):444-55. doi: 10.1016/0003-9861(81)90531-2.
2
Correction of the enzymic defect in cultured fibroblasts from patients with Fabry's disease: treatment with purified alpha-galactosidase from ficin.法布里病患者培养成纤维细胞中酶缺陷的纠正：用无花果蛋白酶纯化的α-半乳糖苷酶治疗
Pediatr Res. 1973 Aug;7(8):684-90. doi: 10.1203/00006450-197308000-00002.
3
Metabolism of ceramide-containing endocytotic vesicles in human diploid fibroblasts.人二倍体成纤维细胞中含神经酰胺的内吞小泡的代谢
J Biol Chem. 1982 Mar 25;257(6):3039-44.
4
Interfacial regulation of acid ceramidase activity. Stimulation of ceramide degradation by lysosomal lipids and sphingolipid activator proteins.酸性神经酰胺酶活性的界面调节。溶酶体脂质和鞘脂激活蛋白对神经酰胺降解的刺激作用。
J Biol Chem. 2001 Feb 23;276(8):5760-8. doi: 10.1074/jbc.M006846200. Epub 2000 Dec 4.
5
The in situ degradation of ceramide, a potential lipid mediator, is not completely impaired in Farber disease.神经酰胺是一种潜在的脂质介质，其原位降解在法伯病中并未完全受损。
FEBS Lett. 1993 Aug 30;329(3):306-12. doi: 10.1016/0014-5793(93)80243-n.
6
Sulfatide and sphingomyelin loading of living cells as tools for the study of ceramide turnover by lysosomal ceramidase--implications for the diagnosis of Farber disease.将硫苷脂和鞘磷脂载入活细胞作为通过溶酶体神经酰胺酶研究神经酰胺周转的工具——对法伯病诊断的意义
Biochem Mol Med. 1995 Apr;54(2):117-25. doi: 10.1006/bmme.1995.1017.
7
Fabry's disease: differentiation between two forms of -galactosidase by myoinositol.法布里病：通过肌醇区分两种形式的α-半乳糖苷酶。
Science. 1972 Aug 11;177(4048):527-8. doi: 10.1126/science.177.4048.527.
8
The role of ceramide in receptor- and stress-induced apoptosis studied in acidic ceramidase-deficient Farber disease cells.在酸性神经酰胺酶缺陷型法伯病细胞中研究神经酰胺在受体和应激诱导的细胞凋亡中的作用。
Oncogene. 2001 Oct 4;20(45):6493-502. doi: 10.1038/sj.onc.1204841.
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Studies on galactosyl ceramide and lactosyl ceramide beta-galactosidase.
Chem Phys Lipids. 1974 Dec;13(4):327-9. doi: 10.1016/0009-3084(74)90007-3.
10
Farber lipogranulomatosis: an unusual presentation in a black child.法伯脂肪肉芽肿病：一名黑人儿童的罕见表现。
Pediatr Neurol. 1986 Nov-Dec;2(6):371-4. doi: 10.1016/0887-8994(86)90082-2.

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Construction of conditional acid ceramidase knockout mice and in vivo effects on oocyte development and fertility.条件性酸性神经酰胺酶基因敲除小鼠的构建及其对卵母细胞发育和生育能力的体内影响。
Cell Physiol Biochem. 2012;30(3):735-48. doi: 10.1159/000341453. Epub 2012 Aug 1.
3
A simple fluorogenic method for determination of acid ceramidase activity and diagnosis of Farber disease.
一种用于测定酸性神经酰胺酶活性和法伯病诊断的简单荧光法。
J Lipid Res. 2010 Dec;51(12):3542-7. doi: 10.1194/jlr.D010033. Epub 2010 Sep 24.
4
Ceramide accumulation is associated with increased apoptotic cell death in cultured fibroblasts of sphingolipid activator protein-deficient mouse but not in fibroblasts of patients with Farber disease.神经酰胺蓄积与鞘脂激活蛋白缺陷小鼠培养成纤维细胞中凋亡性细胞死亡增加有关，但与法伯病患者的成纤维细胞无关。
J Inherit Metab Dis. 1999 Jun;22(5):649-62. doi: 10.1023/a:1005590316064.
5
High-resolution loading tests in the study of genetic heterogeneity in gangliosidosis fibroblasts.神经节苷脂贮积症成纤维细胞遗传异质性研究中的高分辨率负荷试验
J Inherit Metab Dis. 1994;17(1):104-11. doi: 10.1007/BF00735405.
6
A family with combined Farber and Sandhoff, isolated Sandhoff and isolated fetal Farber disease: postnatal exclusion and prenatal diagnosis of Farber disease using lipid loading tests on intact cultured cells.
Eur J Pediatr. 1995 Aug;154(8):643-8. doi: 10.1007/BF02079069.
7
Diagnosis of metachromatic leukodystrophy, Krabbe disease, and Farber disease after uptake of fatty acid-labeled cerebroside sulfate into cultured skin fibroblasts.在脂肪酸标记的硫酸脑苷脂被培养的皮肤成纤维细胞摄取后，对异染性脑白质营养不良、克拉伯病和法伯病进行诊断。
J Clin Invest. 1982 Jul;70(1):89-97. doi: 10.1172/jci110607.
8
Sphingolipid metabolism in cultured fibroblasts: microscopic and biochemical studies employing a fluorescent ceramide analogue.培养的成纤维细胞中的鞘脂代谢：使用荧光神经酰胺类似物的显微镜和生化研究。
Proc Natl Acad Sci U S A. 1983 May;80(9):2608-12. doi: 10.1073/pnas.80.9.2608.
9
Farber's disease in two siblings, sural nerve and subcutaneous biopsies by light and electron microscopy.
Acta Neuropathol. 1986;72(2):178-88. doi: 10.1007/BF00685981.
10
Farber disease: an ultrastructural study. Report of a case and review of the literature.法伯病：超微结构研究。1例报告并文献复习。
Virchows Arch A Pathol Anat Histopathol. 1992;420(3):281-90. doi: 10.1007/BF01600282.