• 文献检索
  • 文档翻译
  • 深度研究
  • 学术资讯
  • Suppr Zotero 插件Zotero 插件
  • 邀请有礼
  • 套餐&价格
  • 历史记录
应用&插件
Suppr Zotero 插件Zotero 插件浏览器插件Mac 客户端Windows 客户端微信小程序
定价
高级版会员购买积分包购买API积分包
服务
文献检索文档翻译深度研究API 文档MCP 服务
关于我们
关于 Suppr公司介绍联系我们用户协议隐私条款
关注我们

Suppr 超能文献

核心技术专利：CN118964589B侵权必究
粤ICP备2023148730 号-1Suppr @ 2026

文献检索

告别复杂PubMed语法，用中文像聊天一样搜索，搜遍4000万医学文献。AI智能推荐，让科研检索更轻松。

立即免费搜索

文件翻译

保留排版，准确专业，支持PDF/Word/PPT等文件格式，支持 12+语言互译。

免费翻译文档

深度研究

AI帮你快速写综述，25分钟生成高质量综述，智能提取关键信息，辅助科研写作。

立即免费体验

The metabolism of sphingomyelin. II. Evidence of an enzymatic deficiency in Niemann-Pick diseae.

作者信息

Brady R O, Kanfer J N, Mock M B, Fredrickson D S

出版信息

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

DOI:10.1073/pnas.55.2.366
PMID:5220952
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC224150/
Abstract
摘要

相似文献

1
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.
2
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.
3
Metabolism of sphingomyelin by intact cultured fibroblasts: differentiation of Niemann-Pick disease type A and B.完整培养的成纤维细胞对鞘磷脂的代谢：A型和B型尼曼-匹克病的鉴别
Biochem Biophys Res Commun. 1982 Mar 15;105(1):14-9. doi: 10.1016/s0006-291x(82)80004-1.
4
Sphingomyelinase in normal human spleens and in spleens from subjects with Niemann-Pick disease.正常人脾脏及尼曼-匹克病患者脾脏中的鞘磷脂酶
J Lipid Res. 1967 May;8(3):202-9.
5
Stimulation of sphingomyelin hydrolysis by cannabidiol in fibroblasts from a Niemann-Pick patient.大麻二酚对尼曼-匹克病患者成纤维细胞中鞘磷脂水解的刺激作用。
Biochem Biophys Res Commun. 1984 May 31;121(1):168-73. doi: 10.1016/0006-291x(84)90702-2.
6
Evidence for both endogenous and exogenous sources of the sphingomyelin storage in lymphoid cell lines from patients with Niemann-Pick disease types A and B.来自A型和B型尼曼-匹克病患者的淋巴细胞系中鞘磷脂储存的内源性和外源性来源的证据。
J Inherit Metab Dis. 1988;11(2):151-7. doi: 10.1007/BF01799864.
7
[Niemann-Pick disease].尼曼-匹克病
Tanpakushitsu Kakusan Koso. 1988 Apr;33(5):738-40.
8
In situ degradation of sphingomyelin by cultured normal fibroblasts and fibroblasts from patients with Niemann-Pick disease type A and C.培养的正常成纤维细胞以及来自A型和C型尼曼-匹克病患者的成纤维细胞对鞘磷脂的原位降解
Biochem Biophys Res Commun. 1982 Oct 15;108(3):1101-6. doi: 10.1016/0006-291x(82)92113-1.
9
A fluorescence-based, high-throughput sphingomyelin assay for the analysis of Niemann-Pick disease and other disorders of sphingomyelin metabolism.一种基于荧光的高通量鞘磷脂测定法，用于分析尼曼-匹克病和其他鞘磷脂代谢紊乱。
Anal Biochem. 2002 Jul 1;306(1):115-23. doi: 10.1006/abio.2002.5686.
10
Biochemical studies in sphingomyelin storage disorders.
Adv Exp Med Biol. 1978;101:731-43. doi: 10.1007/978-1-4615-9071-2_68.

引用本文的文献

1
Outcome of enzyme replacement therapy for hematological and visceral manifestations in children with acid sphingomyelinase deficiency: a single center experience in upper Egypt.酸性鞘磷脂酶缺乏症患儿血液学和内脏表现的酶替代疗法结果：上埃及的单中心经验
Mol Cell Pediatr. 2025 Aug 14;12(1):11. doi: 10.1186/s40348-025-00199-9.
2
Exploring Exhaled Breath Analysis in Adults With Chronic Visceral Acid Sphingomyelinase Deficiency to Identify Potential Biomarkers of Pulmonary Involvement.探索慢性内脏酸性鞘磷脂酶缺乏症成人患者的呼出气分析，以确定肺部受累的潜在生物标志物。
J Inherit Metab Dis. 2025 Jul;48(4):e70039. doi: 10.1002/jimd.70039.
3
Lysosomal Repair in Health and Disease.健康与疾病中的溶酶体修复
J Cell Physiol. 2025 May;240(5):e70044. doi: 10.1002/jcp.70044.
4
Liver magnetic resonance spectroscopy as an alternative for evaluating Niemann-Pick C disease progression.肝脏磁共振波谱分析作为评估尼曼-匹克C型病进展的一种替代方法。
RSC Adv. 2025 Feb 6;15(6):4079-4085. doi: 10.1039/d4ra06781a.
5
Acid sphingomyelinase deficiency and Gaucher disease in adults: Similarities and differences in two macrophage storage disorders.成人酸性鞘磷脂酶缺乏症和戈谢病：两种巨噬细胞贮积症的异同
JIMD Rep. 2024 Jul 4;65(5):330-340. doi: 10.1002/jmd2.12420. eCollection 2024 Sep.
6
Phospholipids, Sphingolipids, and Cholesterol-Derived Lipid Mediators and Their Role in Neurological Disorders.磷脂、鞘脂和胆固醇衍生的脂质介质及其在神经疾病中的作用。
Int J Mol Sci. 2024 Oct 3;25(19):10672. doi: 10.3390/ijms251910672.
7
Natural disease course of chronic visceral acid sphingomyelinase deficiency in adults: A first step toward treatment criteria.成人慢性内脏酸性鞘磷脂酶缺乏症的自然病程：迈向治疗标准的第一步。
J Inherit Metab Dis. 2025 Jan;48(1):e12789. doi: 10.1002/jimd.12789. Epub 2024 Aug 23.
8
Sphingolipids in mitochondria-from function to disease.线粒体中的鞘脂——从功能到疾病
Front Cell Dev Biol. 2023 Nov 21;11:1302472. doi: 10.3389/fcell.2023.1302472. eCollection 2023.
9
Glycosphingolipids are linked to elevated neurotransmission and neurodegeneration in a Drosophila model of Niemann Pick type C.鞘糖脂与尼曼-皮克 C 型果蝇模型中神经传递和神经退行性变的升高有关。
Dis Model Mech. 2023 Oct 1;16(10). doi: 10.1242/dmm.050206. Epub 2023 Oct 12.
10
A Brief Overview of the Toxic Sphingomyelinase Ds of Brown Recluse Spider Venom and Other Organisms and Simple Methods To Detect Production of Its Signature Cyclic Ceramide Phosphate.褐隐士蜘蛛毒液及其他生物中的毒性鞘磷脂酶Ds概述以及检测其标志性环磷酸神经酰胺产生的简单方法。
Mol Pharmacol. 2024 Feb 15;105(3):144-154. doi: 10.1124/molpharm.123.000709.

本文引用的文献

1
Phosphorylcholine in rat tissues.大鼠组织中的磷酰胆碱。
Biochem J. 1955 Jun;60(2):325-8. doi: 10.1042/bj0600325.
2
METABOLISM OF GLUCOCEREBROSIDES. II. EVIDENCE OF AN ENZYMATIC DEFICIENCY IN GAUCHER'S DISEASE.葡萄糖脑苷脂的代谢。II. 戈谢病中酶缺乏的证据。
Biochem Biophys Res Commun. 1965 Jan 18;18:221-5. doi: 10.1016/0006-291x(65)90743-6.
3
Rapid sensitive method for determining H3-water in body fluids by liquid scintillation spectrometry.通过液体闪烁光谱法测定体液中H3-水的快速灵敏方法。
Proc Soc Exp Biol Med. 1959 Oct;102:8-12. doi: 10.3181/00379727-102-25125.
4
Sphingomyelin synthesis in Niemann-Pick disease.尼曼-匹克病中的鞘磷脂合成
Am J Clin Nutr. 1961 Jan-Feb;9:63-7. doi: 10.1093/ajcn/9.1.63.