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Rabbit Anti-GLDC  antibody (bs-13370R)  
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-13370R
英文名称 Rabbit Anti-GLDC  antibody
中文名称 甘氨酸脱羧酶P蛋白抗体
别    名 GCE; GCSP; GCSP_HUMAN; GLDC; Glycine cleavage system P protein; glycine cleavage system protein P; Glycine decarboxylase; glycine decarboxylase P protein; Glycine dehydrogenase (decarboxylating) mitochondrial; Glycine dehydrogenase [decarboxylating], mitochondrial; Gycine dehydrogenase (decarboxylating); HYGN1; MGC138198; MGC138200; NKH.  
研究领域 肿瘤  细胞生物  信号转导  激酶和磷酸酶  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human,Mouse,Rat,Rabbit,Dog,Horse)
产品应用 ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 109kDa
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GLDC: 51-150/1020 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
产品介绍 The glycine cleavage system is comprised of AMT (known as Protein T), GCSH (known as Protein H), DLD (known as Protein L) and GLDC (known as Protein P), all of which work together to catalyze the cleavage and degradation of glycine. GLDC (glycine dehydrogenase ), also known as GCE, GCSP (glycine cleavage system P protein) or HYGN1, is a 1,020 amino acid protein that localizes to the mitochondria and belongs to the gcvP family. GLDC binds to glycine and enables the methylamine group from glycine to be transferred to the Protein T. GLDC exists as a homodimer and utilizes pyridoxal phosphate as a cofactor. Mutations in the gene encoding GLDC leads to nonketotic hyperglycinemia (NKH), also known as glycine encephalopathy (GCE), an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.

Function:
The glycine cleavage system catalyzes the degradation of glycine. The P protein binds the alpha-amino group of glycine through its pyridoxal phosphate cofactor; CO(2) is released and the remaining methylamine moiety is then transferred to the lipoamide cofactor of the H protein.

Subunit:
Homodimer. The glycine cleavage system is composed of four proteins: P, T, L and H.

Subcellular Location:
Mitochondrion.

DISEASE:
Defects in GLDC are a cause of non-ketotic hyperglycinemia (NKH) [MIM:605899]; also known as glycine encephalopathy (GCE). NKH is an autosomal recessive disease characterized by accumulation of a large amount of glycine in body fluid and by severe neurological symptoms.

Similarity:
Belongs to the gcvP family.

SWISS:
P23378

Gene ID:
2731

Database links:

Entrez Gene: 2731 Human

Entrez Gene: 104174 Mouse

Omim: 238300 Human

SwissProt: P23378 Human

SwissProt: Q91W43 Mouse

Unigene: 584238 Human

Unigene: 274852 Mouse



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