A lysosomal storage disorder in mice: a model of Niemann-Pick disease.

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作者信息

Sakiyama T, Tsuda M, Kitagawa T, Fujita R, Miyawaki S

出版信息

J Inherit Metab Dis. 1982;5(4):239-40. doi: 10.1007/BF02179154.

DOI:10.1007/BF02179154

PMID:6820448

Abstract

摘要

相似文献

A lysosomal storage disorder in mice: a model of Niemann-Pick disease.

J Inherit Metab Dis. 1982;5(4):239-40. doi: 10.1007/BF02179154.

[Study of acid sphingomyelinase in Niemann-Pick mice].[尼曼-匹克病小鼠酸性鞘磷脂酶的研究]

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Niemann-Pick C disease: cystine and lipids accumulate in the murine model of this lysosomal cholesterol lipidosis.尼曼-皮克C型病：在这种溶酶体胆固醇脂质贮积症的小鼠模型中，胱氨酸和脂质会蓄积。

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Subcellular localization of acid sphingomyelinase and lipid in Niemann-Pick mice.尼曼-匹克病小鼠中酸性鞘磷脂酶和脂质的亚细胞定位

J Inherit Metab Dis. 1987;10(3):301-4. doi: 10.1007/BF01800087.

Bone marrow transplantation for Niemann-Pick mice.

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Pulmonary delivery of recombinant acid sphingomyelinase improves clearance of lysosomal sphingomyelin from the lungs of a murine model of Niemann-Pick disease.重组酸性鞘磷脂酶的肺部给药可改善尼曼-匹克病小鼠模型肺部溶酶体鞘磷脂的清除。

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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

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Niemann-Pick type C disease and intracellular cholesterol trafficking.尼曼-匹克C型病与细胞内胆固醇转运

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Lipid storage disorders block lysosomal trafficking by inhibiting a TRP channel and lysosomal calcium release.脂质贮积症通过抑制 TRP 通道和溶酶体钙释放来阻断溶酶体运输。

Nat Commun. 2012 Mar 13;3:731. doi: 10.1038/ncomms1735.

Concurrent increase of cholesterol, sphingomyelin and glucosylceramide in the spleen from non-neurologic Niemann-Pick type C patients but also patients possibly affected with other lipid trafficking disorders.非神经型尼曼-匹克C型患者以及可能患有其他脂质转运障碍的患者脾脏中胆固醇、鞘磷脂和葡糖神经酰胺同时增加。

FEBS Lett. 2003 Feb 27;537(1-3):177-81. doi: 10.1016/s0014-5793(03)00100-5.

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A simple methodology to assess endolysosomal protease activity involved in antigen processing in human primary cells.一种评估参与人类原代细胞抗原加工的内溶酶体蛋白酶活性的简单方法。

BMC Cell Biol. 2013 Aug 9;14:35. doi: 10.1186/1471-2121-14-35.

Cholesterol and oxygenated cholesterol concentrations are markedly elevated in peripheral tissue but not in brain from mice with the Niemann-Pick type C phenotype.在具有尼曼-匹克C型表型的小鼠中，外周组织中的胆固醇和氧化胆固醇浓度显著升高，但脑内则不然。

J Inherit Metab Dis. 1998 Dec;21(8):853-63. doi: 10.1023/a:1005474803278.

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Type C Niemann-Pick disease in a boxer dog.

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Restoration of the cholesterol metabolism in 3T3 cell lines derived from the sphingomyelinosis mouse (spm/spm) by transfer of a human chromosome 18.通过转移人类18号染色体恢复源自鞘磷脂沉积病小鼠（spm/spm）的3T3细胞系中的胆固醇代谢。

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Bone marrow transplantation for Niemann-Pick mice.尼曼-匹克病小鼠的骨髓移植

J Inherit Metab Dis. 1983;6(3):129-30. doi: 10.1007/BF01800746.

Bone marrow transplantation in Niemann-Pick mice.尼曼-匹克病小鼠的骨髓移植

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Lack of acid sphingomyelinase in the mitochondria-lysosome fraction of brain from Niemann-Pick mice.尼曼-匹克病小鼠大脑线粒体-溶酶体部分中酸性鞘磷脂酶的缺失。

J Inherit Metab Dis. 1985;8(3):147-8. doi: 10.1007/BF01819301.

Subcellular localization of acid sphingomyelinase and lipid in Niemann-Pick mice.尼曼-匹克病小鼠中酸性鞘磷脂酶和脂质的亚细胞定位

J Inherit Metab Dis. 1987;10(3):301-4. doi: 10.1007/BF01800087.

Feline sphingolipidosis resembling Niemann-Pick disease type C.类似尼曼-匹克病C型的猫鞘脂沉积症

Acta Neuropathol. 1990;81(2):189-97. doi: 10.1007/BF00334507.

本文引用的文献

Protein measurement with the Folin phenol reagent.使用福林酚试剂进行蛋白质测定。

J Biol Chem. 1951 Nov;193(1):265-75.

FORM-CELL RETICULOSIS OF MICE: AN INHERITED CONDITION RESEMBLING GAUCHER'S AND NIERMANN-PICK DISEASES.小鼠的泡沫细胞网状细胞增多症：一种类似高雪氏病和尼曼-皮克病的遗传性疾病。

J Med Genet. 1965 Jun;2(2):99-106. doi: 10.1136/jmg.2.2.99.

Simple rapid microtechnic for serum total cholesterol.血清总胆固醇简易快速微量技术

Am J Clin Pathol. 1957 May;27(5):583-8. doi: 10.1093/ajcp/27.5_ts.583.

A simple method for the isolation and purification of total lipides from animal tissues.一种从动物组织中分离和纯化总脂质的简单方法。

J Biol Chem. 1957 May;226(1):497-509.

Sphingomyelinosis, a new mutation in the mouse: a model of Niemann-Pick disease in humans.鞘磷脂沉积症，小鼠中的一种新突变：人类尼曼-匹克病的模型。

J Hered. 1982 Jul-Aug;73(4):257-63. doi: 10.1093/oxfordjournals.jhered.a109635.

The metabolism of sphingomyelin. I. Purification and properties of a sphingomyelin-cleaving enzyme from rat liver tissue.鞘磷脂的代谢。I. 从大鼠肝脏组织中纯化鞘磷脂裂解酶及其性质

J Biol Chem. 1966 Mar 10;241(5):1081-4.

Lipid storage disease in a Siamese cat.一只暹罗猫患脂质贮积病。

J Am Vet Med Assoc. 1970 Mar 1;156(5):616-22.

A practical chromogenic procedure for the detection of homozygotes and heterozygous carriers of Niemann-Pick disease.一种用于检测尼曼-匹克病纯合子和杂合子携带者的实用显色方法。

N Engl J Med. 1975 Sep 25;293(13):632-6. doi: 10.1056/NEJM197509252931304.

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