源自尼曼-皮克病患者的组织培养物中鞘磷脂裂解酶活性缺乏。 - Suppr

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超能文献

Deficiency of sphingomyelin-cleaving enzyme activity in tissue cultures derived from patients with Niemann-Pick disease.

作者信息

Sloan H R, Uhlendorf B W, Kanfer J N, Brady R O, Fredrickson D S

出版信息

Biochem Biophys Res Commun. 1969 Mar 10;34(5):582-8. doi: 10.1016/0006-291x(69)90777-3.

DOI:10.1016/0006-291x(69)90777-3

PMID:5777777

Abstract

摘要

相似文献

Deficiency of sphingomyelin-cleaving enzyme activity in tissue cultures derived from patients with Niemann-Pick disease.源自尼曼-皮克病患者的组织培养物中鞘磷脂裂解酶活性缺乏。

Biochem Biophys Res Commun. 1969 Mar 10;34(5):582-8. doi: 10.1016/0006-291x(69)90777-3.

Tissue culture studies in the lipid storage disorders.脂质贮积病的组织培养研究

Chem Phys Lipids. 1970 Oct;5(1):250-60. doi: 10.1016/0009-3084(70)90022-8.

A family with visceral course of Niemann-Pick disease, macular halo syndrome and low sphingomyelin degradation rate.一个患有尼曼-匹克病内脏型、黄斑晕轮综合征且鞘磷脂降解率低的家族。

J Inherit Metab Dis. 1994;17(1):93-103. doi: 10.1007/BF00735404.

Metabolism of sphingomyelin in cultured skin fibroblasts from patients with different types of Niemann-Pick disease.不同类型尼曼-匹克病患者培养皮肤成纤维细胞中鞘磷脂的代谢

Tohoku J Exp Med. 1986 Apr;148(4):365-71. doi: 10.1620/tjem.148.365.

Uptake and metabolism of radioactively labeled sphingomyelin in cultured skin fibroblasts from controls and patients with Niemann-Pick disease and other lysosomal storage diseases.来自对照个体以及患有尼曼-匹克病和其他溶酶体贮积病患者的培养皮肤成纤维细胞中放射性标记鞘磷脂的摄取与代谢

Biochim Biophys Acta. 1983 Nov 1;754(1):82-92. doi: 10.1016/0005-2760(83)90084-x.

The metabolism of sphingomyelin. II. Evidence of an enzymatic deficiency in Niemann-Pick diseae.鞘磷脂的代谢。II. 尼曼-匹克病中酶缺乏的证据。

Proc Natl Acad Sci U S A. 1966 Feb;55(2):366-9. doi: 10.1073/pnas.55.2.366.

Clinical and biochemical diagnostics of Niemann-Pick disease.尼曼-匹克病的临床与生化诊断

Klin Padiatr. 1986 Mar-Apr;198(2):103-6. doi: 10.1055/s-2008-1026862.

Beta-galactosidase in tissue culture derived from human skin and bone marrow. Enzyme defect in GM1 gangliosidosis.源自人皮肤和骨髓的组织培养中的β-半乳糖苷酶。GM1神经节苷脂贮积症中的酶缺陷。

Pediatr Res. 1969 Nov;3(6):532-7. doi: 10.1203/00006450-196911000-00002.

Studies on the activation of sphingomyelinase activity in Niemann-Pick type A, B, and C fibroblasts: enzymological differentiation of types A and B.关于尼曼-匹克病A型、B型和C型成纤维细胞中鞘磷脂酶活性激活的研究：A型和B型的酶学鉴别

Pediatr Res. 1984 Nov;18(11):1088-93. doi: 10.1203/00006450-198411000-00006.

Enzymatic hydrolysis of sphingomyelin liposomes by normal tissues and tissues from patients with Niemann-Pick disease.正常组织和尼曼-匹克病患者组织对鞘磷脂脂质体的酶促水解作用。

Biochim Biophys Acta. 1983 May 16;751(3):428-31. doi: 10.1016/0005-2760(83)90302-8.

引用本文的文献

Understanding and Treating Niemann-Pick Type C Disease: Models Matter.理解和治疗尼曼-匹克 C 型疾病：模型很重要。

Int J Mol Sci. 2020 Nov 26;21(23):8979. doi: 10.3390/ijms21238979.

Recent advances in elucidating Niemann-Pick C disease.尼曼-皮克C型病研究的最新进展

Brain Pathol. 1998 Jan;8(1):163-74. doi: 10.1111/j.1750-3639.1998.tb00143.x.

The genetic mismanagement of complex lipid metabolism.复杂脂质代谢的基因管理不当。

Bull N Y Acad Med. 1971 Feb;47(2):173-82.

Enzyme deficiency in cholesteryl ester storage idisease.胆固醇酯贮积病中的酶缺乏

J Clin Invest. 1972 Jul;51(7):1923-6. doi: 10.1172/JCI106997.

In utero diagnosis of Niemann-Pick disease.尼曼-匹克病的宫内诊断

Am J Hum Genet. 1971 Sep;23(5):533-5.

Niemann-pick variant disorders: comparison of errors of cellular cholesterol homeostasis in group D and group C fibroblasts.尼曼-匹克变异型疾病：D组和C组成纤维细胞中细胞胆固醇稳态异常的比较

Proc Natl Acad Sci U S A. 1987 Jan;84(2):556-60. doi: 10.1073/pnas.84.2.556.

Experimental polymer storage disease in rabbits. An approach to the histogenesis of sphingolipidoses.兔实验性聚合物贮积病。鞘脂类贮积症组织发生学的研究方法。

Virchows Arch A Pathol Anat Histol. 1975;365(4):351-65. doi: 10.1007/BF00471182.

Studies on sphingomyelinase activity in cultured cells and leucocytes.培养细胞和白细胞中鞘磷脂酶活性的研究。

J Inherit Metab Dis. 1978;1(1):29-33. doi: 10.1007/BF01805712.

Sphingomyelinase activities in cultured skin fibroblasts from patients with Niemann-Pick Disease.尼曼-匹克病患者培养的皮肤成纤维细胞中的鞘磷脂酶活性。

Hum Genet. 1979 Mar 12;47(2):159-67. doi: 10.1007/BF00273198.

A new form of Niemann-Pick disease characterised by temperature-labile sphingomyelinase.一种以温度不稳定的鞘磷脂酶为特征的新型尼曼-匹克病。

J Med Genet. 1978 Oct;15(5):370-4. doi: 10.1136/jmg.15.5.370.

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