Anti-MMACHC抗体-抗体-抗体-生物在线
Anti-MMACHC抗体

Anti-MMACHC抗体

商家询价

产品名称: Anti-MMACHC抗体

英文名称: MMACHC

产品编号: YB--18960R

产品价格: null

产品产地: 中国/美国

品牌商标: Ybscience

更新时间: 2023-08-17T10:29:50

使用范围: 科研使用

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 Anti-MMACHC抗体

产品编号 YB-18960R
英文名称 MMACHC
中文名称 甲基丙二酸尿症和高胱氨酸尿症C型蛋白同源抗体
别    名 1810037K07Rik; BOS_3654; cblC; DKFZp564I122; FLJ25671; Methylmalonic aciduria (cobalamin deficiency) cblC type with homocystinuria; Methylmalonic aciduria and homocystinuria type C protein; Methylmalonic aciduria and homocystinuria type C protein homolog; MGC134307; MMAC_HUMAN; MMACHC; OTTHUMP00000009243; RP11 291L19.3; RP23-177C18.3.  
规格价格 100ul/1580元 购买    200ul/2480元 购买    大包装/询价
说 明 书 100ul  200ul
研究领域 细胞生物  免疫学  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Dog, Cow, Horse, Sheep, 
产品应用 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 32kDa
细胞定位 细胞浆 
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human MMACHC:151-250/282 
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
PubMed PubMed
产品介绍 background:
The exact function of the protein encoded by this gene is not known, however, its C-terminal region shows similarity to TonB, a bacterial protein involved in energy transduction for cobalamin (vitamin B12) uptake. Hence, it is postulated that this protein may have a role in the binding and intracellular trafficking of cobalamin. Mutations in this gene are associated with methylmalonic aciduria and homocystinuria type cblC. [provided by RefSeq, Oct 2009]

Function:
May be involved in the binding and intracellular trafficking of cobalamin (vitamin B12).

Tissue Specificity:
Widely expressed. Expressed at higher level in fetal liver. Also expressed in spleen, lymph node, thymus and bone marrow. Weakly or not expressed in peripheral blood leukocytes.

DISEASE:
Defects in MMACHC are the cause of methylmalonic aciduria and homocystinuria type cblC (MMACHC) [MIM:277400]. 
MMACHC is a disorder of cobalamin metabolism characterized by decreased levels of the coenzymes adenosylcobalamin (AdoCbl) and methylcobalamin (MeCbl). Affected individuals may have developmental, hematologic, neurologic, metabolic, ophthalmologic, and dermatologic clinical findings. Although considered a disease of infancy or childhood, some individuals develop symptoms in adulthood.

Similarity:
Belongs to the MMACHC family.

SWISS:
Q9Y4U1

Gene ID:
25974

Database links:

Entrez Gene: 513433 Cow

Entrez Gene: 25974 Human

Entrez Gene: 67096 Mouse

Entrez Gene: 313520 Rat

Omim: 609831 Human

SwissProt: Q5E9C8 Cow

SwissProt: Q9Y4U1 Human

SwissProt: Q9CZD0 Mouse

Unigene: 13024 Human



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