磷脂酶活性降低、肽储存与犬神经元蜡样脂褐质沉积症的发病机制 - Suppr

Suppr

超能文献

Reduced phospholipase activity, peptide storage and the pathogenesis of canine neuronal ceroid-lipofuscinosis.

作者信息

Dawson G, Kilkus J, Siakotos A N

机构信息

Department of Pediatrics, University of Chicago, IL 60637.

出版信息

J Inherit Metab Dis. 1993;16(2):330-4. doi: 10.1007/BF00710276.

DOI:10.1007/BF00710276

PMID:8411992

Abstract

摘要

相似文献

Reduced phospholipase activity, peptide storage and the pathogenesis of canine neuronal ceroid-lipofuscinosis.磷脂酶活性降低、肽储存与犬神经元蜡样脂褐质沉积症的发病机制

J Inherit Metab Dis. 1993;16(2):330-4. doi: 10.1007/BF00710276.

Phospholipases and the molecular basis for the formation of ceroid in Batten disease.磷脂酶与巴滕病中蜡样质形成的分子基础。

Adv Exp Med Biol. 1989;266:259-70; discussion 271. doi: 10.1007/978-1-4899-5339-1_18.

Lysosomal degradation of cholecystokinin-(29-33)-amide in mouse brain is dependent on tripeptidyl peptidase-I: implications for the degradation and storage of peptides in classical late-infantile neuronal ceroid lipofuscinosis.小鼠脑中胆囊收缩素 -（29 - 33）- 酰胺的溶酶体降解依赖于三肽基肽酶 -I：对经典晚发性婴儿神经元蜡样脂褐质沉积症中肽类降解和储存的影响

Biochem J. 2002 Sep 1;366(Pt 2):521-9. doi: 10.1042/BJ20020467.

Palmitate oxidation in muscle mitochondria of patients with the juvenile form of neuronal ceroid-lipofuscinosis.青少年型神经元蜡样脂褐质沉积症患者肌肉线粒体中的棕榈酸氧化

Am J Med Genet. 1995 Jun 5;57(2):298-300. doi: 10.1002/ajmg.1320570237.

Methylated lysine in storage body protein of sheep with hereditary ceroid-lipofuscinosis.患有遗传性类脂褐质沉积症的绵羊储存体蛋白中的甲基化赖氨酸

Biochim Biophys Acta. 1992 Feb 14;1138(2):97-108. doi: 10.1016/0925-4439(92)90048-r.

Analysis of dolichyl pyrophosphoryl oligosaccharides in purified storage cytosomes from ovine ceroid-lipofuscinosis.绵羊蜡样质脂褐质沉积症纯化储存胞质小体中多萜醇焦磷酸寡糖的分析

Biochim Biophys Acta. 1989 Dec 8;993(2-3):245-51. doi: 10.1016/0304-4165(89)90172-4.

Abnormal processing of carboxy-terminal fragment of beta precursor protein (beta PP) in neuronal ceroid-lipofuscinosis (NCL) cases.神经元蜡样脂褐质沉积症（NCL）病例中β-前体蛋白（β-PP）羧基末端片段的异常加工。

J Inherit Metab Dis. 1993;16(2):312-6. doi: 10.1007/BF00710273.

Enzymatic and molecular biological analysis of palmitoyl protein thioesterase deficiency in infantile neuronal ceroid lipofuscinosis.

J Neurochem. 1998 Jul;71(1):323-9. doi: 10.1046/j.1471-4159.1998.71010323.x.

A rare homozygous MFSD8 single-base-pair deletion and frameshift in the whole genome sequence of a Chinese Crested dog with neuronal ceroid lipofuscinosis.一只患有神经元蜡样脂褐质沉积症的中国冠毛犬全基因组序列中罕见的纯合子MFSD8单碱基对缺失和移码突变。

BMC Vet Res. 2015 Jan 3;10:960. doi: 10.1186/s12917-014-0181-z.

Regulation of the hydrolysis of phosphatidylcholine in Swiss 3T3 cells.瑞士3T3细胞中磷脂酰胆碱水解的调控

Biochem Soc Trans. 1991 Apr;19(2):321-4. doi: 10.1042/bst0190321.

本文引用的文献

Storage bodies in the ceroid-lipofuscinoses (Batten disease): low-molecular-weight components, unusual amino acids and reconstitution of fluorescent bodies from non-fluorescent components.蜡样脂褐质沉积症（巴滕病）中的储存小体：低分子量成分、异常氨基酸以及由非荧光成分重构荧光小体

J Inherit Metab Dis. 1993;16(2):292-5. doi: 10.1007/BF00710268.

Ceroid, lipofuscin and the ceroid-lipofuscinoses (Batten disease).脂褐素、脂褐质与脂褐质沉积症（巴滕病）

J Inherit Metab Dis. 1993;16(2):280-3. doi: 10.1007/BF00710265.

Evidence for two distinct forms of mammalian beta-mannosidase.哺乳动物β-甘露糖苷酶两种不同形式的证据。

J Biol Chem. 1982 Apr 10;257(7):3369-71.

The large-scale separation of peroxisomes, mitochondria, and lysosomes from the livers of rats injected with triton WR-1339. Improved isolation procedures, automated analysis, biochemical and morphological properties of fractions.从注射曲拉通WR-1339的大鼠肝脏中大规模分离过氧化物酶体、线粒体和溶酶体。改进的分离程序、自动分析、各组分的生化和形态学特性。

J Cell Biol. 1968 May;37(2):482-513. doi: 10.1083/jcb.37.2.482.

Purification of lysosomal phospholipase A and demonstration of proteins that inhibit phospholipase A in a lysosomal fraction from rat kidney cortex.

Biochemistry. 1986 Oct 21;25(21):6456-61. doi: 10.1021/bi00369a017.

Accumulation of dolichol-linked oligosaccharides in ceroid-lipofuscinosis (Batten disease).

Am J Med Genet Suppl. 1988;5:221-32. doi: 10.1002/ajmg.1320310625.

4-Hydroxynonenal: a specific indicator for canine neuronal-retinal ceroidosis.4-羟基壬烯醛：犬神经元视网膜蜡样脂褐质沉积症的特异性指标。

Am J Med Genet Suppl. 1988;5:171-81. doi: 10.1002/ajmg.1320310620.

Ubiquitin-mediated protein degradation.泛素介导的蛋白质降解

J Biol Chem. 1988 Oct 25;263(30):15237-40.

Ovine ceroid lipofuscinosis. The major lipopigment protein and the lipid-binding subunit of mitochondrial ATP synthase have the same NH2-terminal sequence.绵羊蜡样脂褐质沉积症。主要脂色素蛋白与线粒体ATP合酶的脂质结合亚基具有相同的NH2末端序列。

J Biol Chem. 1989 Apr 5;264(10):5736-40.

Phospholipases and the molecular basis for the formation of ceroid in Batten disease.磷脂酶与巴滕病中蜡样质形成的分子基础。

Adv Exp Med Biol. 1989;266:259-70; discussion 271. doi: 10.1007/978-1-4899-5339-1_18.

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