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Rabbit Anti-GLDC/Gold Conjugated antibody (bs-13370R-Gold)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul(10nm  15nm  35nm
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-13370R-Gold
英文名稱 Rabbit Anti-GLDC/Gold Conjugated antibody
中文名稱 膠體金標記的甘氨酸脫羧酶P蛋白抗體
別    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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul(10nm  15nm  35nm
研究領域 腫瘤  細胞生物  信號轉導  激酶和磷酸酶  新陳代謝  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Horse, Rabbit, )
產(chǎn)品應用 IEM=1:20-200 ICA=1:20-200 ChIP=1:20-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 109kDa
性    狀 Lyophilized or Liquid
濃    度 0.4mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human GLDC
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.02M TBS(pH8.2) with 1% BSA, 0.03% Proclin300.
保存條件 Store at 2-8 oC for 3-6 months. Avoid repeated freeze/thaw cycles.
產(chǎn)品介紹 background:
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.

Database links:
UniProtKB/Swiss-Prot: P23378.2

Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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