• 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

[与甘氨酸和β-丙氨酸相关的物质代谢异常]

[Abnormal metabolism of substances related to glycine and beta-alanine].

作者信息

Hiraga K, Koyata H

机构信息

Department of Biochemistry, Toyama Medical and Pharmaceutical University, School of Medicine.

出版信息

Nihon Rinsho. 1992 Jul;50(7):1575-80.

PMID:1404886
Abstract

Glycine and serine are interconverted by serine hydroxymethyltransferase and hence share their metabolic pathways. In the rat the carbon skeleton of choline is derived from the alpha and beta carbons of serine through phosphatidylserine and is further converted to that of glycine through N-methylated molecules of glycine i.e. betaine, N-dimethylglycine, and sarcosine (N-methylglycine). Thus far, the production of a folate derivative of the active one carbon that is an essential material for purine nucleotides and thymidylate biosynthesis is the notable feature of this metabolic process. In the major degradative pathway for pyrimidine nucleotides, beta-alanine, one of omega-amino acids, is produced and further catabolized by beta-alanine: oxoglutarate transaminase which is suggested to be identical to that for gamma-aminobutyrate, another omega-amino acid. Disorders included in these metabolic pathways are succinctly reviewed.

摘要

甘氨酸和丝氨酸可通过丝氨酸羟甲基转移酶相互转化,因此共享其代谢途径。在大鼠中,胆碱的碳骨架通过磷脂酰丝氨酸源自丝氨酸的α和β碳原子,并通过甘氨酸的N-甲基化分子(即甜菜碱、N-二甲基甘氨酸和肌氨酸(N-甲基甘氨酸))进一步转化为甘氨酸的碳骨架。到目前为止,活性一碳叶酸衍生物的产生是该代谢过程的显著特征,该衍生物是嘌呤核苷酸和胸苷酸生物合成的必需物质。在嘧啶核苷酸的主要降解途径中,产生了ω-氨基酸之一的β-丙氨酸,并通过β-丙氨酸:氧代戊二酸转氨酶进一步分解代谢,该转氨酶被认为与另一种ω-氨基酸γ-氨基丁酸的转氨酶相同。对这些代谢途径中包含的疾病进行了简要综述。

相似文献

1
[Abnormal metabolism of substances related to glycine and beta-alanine].[与甘氨酸和β-丙氨酸相关的物质代谢异常]
Nihon Rinsho. 1992 Jul;50(7):1575-80.
2
[Molecular lesion and pathophysiology of hyperglycinemia: glycine cleavage system, physiology and pathology].[高甘氨酸血症的分子病变与病理生理学:甘氨酸裂解系统、生理学与病理学]
Seikagaku. 1993 Apr;65(4):248-59.
3
Inborn errors of GABA metabolism.γ-氨基丁酸代谢的先天性缺陷。
Bioessays. 1986 Jan;4(1):24-7. doi: 10.1002/bies.950040107.
4
[Hyperbetaalaninemia].[高β-丙氨酸血症]
Ryoikibetsu Shokogun Shirizu. 1998(18 Pt 1):235-7.
5
[Nonketotic hyperglycinemia].[非酮症高甘氨酸血症]
Ryoikibetsu Shokogun Shirizu. 1998(18 Pt 1):227-30.
6
Nonketotic hyperglycinemia: two patients with primary defects of P-protein and T-protein, respectively, in the glycine cleavage system.非酮症高甘氨酸血症:分别有两名患者在甘氨酸裂解系统中存在P蛋白和T蛋白的原发性缺陷。
Pediatr Res. 1983 Dec;17(12):967-70. doi: 10.1203/00006450-198312000-00008.
7
Non-ketotic hyperglycinaemia: clinical and biochemical aspects.
Eur J Pediatr. 1987 May;146(3):221-7. doi: 10.1007/BF00716464.
8
Tracing Metabolic Fate of Mitochondrial Glycine Cleavage System Derived Formate In Vitro and In Vivo.体外和体内追踪线粒体甘氨酸分解系统衍生甲酸的代谢命运。
Int J Mol Sci. 2020 Nov 20;21(22):8808. doi: 10.3390/ijms21228808.
9
Structural requirements for activation of the glycine coagonist site of N-methyl-D-aspartate receptors expressed in Xenopus oocytes.非洲爪蟾卵母细胞中表达的N-甲基-D-天冬氨酸受体甘氨酸共激动剂位点激活的结构要求。
Mol Pharmacol. 1989 Oct;36(4):556-65.
10
[Molecular pathology of the hyperglycinemias].[高甘氨酸血症的分子病理学]
Rev Esp Fisiol. 1982;38 Suppl:199-205.