FITC标记的核输出信号相互作用蛋白抗体-抗体-抗体-生物在线
上海沪震实业有限公司
FITC标记的核输出信号相互作用蛋白抗体

FITC标记的核输出信号相互作用蛋白抗体

商家询价

产品名称: FITC标记的核输出信号相互作用蛋白抗体

英文名称: Anti-LMBRD1/FITC

产品编号: HZ-18309R-FITC

产品价格: null

产品产地: 中国/上海

品牌商标: HZbscience

更新时间: 2023-08-17T10:24:20

使用范围: ICC=1:50-200 IF=1:50-200

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 Rabbit Anti-LMBRD1/FITC Conjugated antibody

FITC标记的核输出信号相互作用蛋白抗体

 

英文名称 Anti-LMBRD1/FITC
中文名称 FITC标记的核输出信号相互作用蛋白抗体
别    名 HDAg-L-interacting protein NESI; LMBD1_HUMAN; LMBR1 domain-containing protein 1; lmbrd1; Nuclear export signal-interacting protein; Probable lysosomal cobalamin transporter.  
规格价格 100ul/2980元 购买        大包装/询价
说 明 书 100ul  
研究领域 肿瘤  细胞生物  免疫学  信号转导  新陈代谢  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit, Sheep, 
产品应用 ICC=1:50-200 IF=1:50-200  
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 61kDa
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human LMBRD1
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.
产品介绍 background:
This gene encodes a lysosomal membrane protein that may be involved in the transport and metabolism of cobalamin. This protein also interacts with the large form of the hepatitis delta antigen and may be required for the nucleocytoplasmic shuttling of the hepatitis delta virus. Mutations in this gene are associated with the vitamin B12 metabolism disorder termed, homocystinuria-megaloblastic anemia complementation type F.[provided by RefSeq, Oct 2009]

Function:
Probable lysosomal cobalamin transporter. Required to export cobalamin from lysosomes allowing its conversion to cofactors. Isoform 3 may play a role in the assembly of hepatitis delta virus (HDV).

Subcellular Location:
Lysosome membrane.

Tissue Specificity:
Isoform 3 is expressed in liver.

Post-translational modifications:
N-glycosylated.

DISEASE:
Defects in LMBRD1 are the cause of methylmalonic aciduria and homocystinuria type cblF (MMAFHC) [MIM:277380]; also known as homocystinuria-megaloblastic anemia complementation type F. MMAFHC is a disorder of cobalamin metabolism characterized by decreased levels of the coenzymes adenosylcobalamin (AdoCbl) and methylcobalamin (MeCbl). It is due to accumulation of free cobalamin in lysosomes, thus hindering its conversion to cofactors. Clinical features include developmental delay, stomatitis, glossitis, seizures and methylmalonic aciduria responsive to vitamin B12.

Similarity:
Belongs to the LIMR family. LMBRD1 subfamily.

Database links:

Entrez Gene: 55788 Human

Entrez Gene: 68421 Mouse

Entrez Gene: 246046 Rat

Omim: 612625 Human

SwissProt: Q9NUN5 Human

SwissProt: Q8K0B2 Mouse

SwissProt: Q6AZ61 Rat

Unigene: 271643 Human

Unigene: 677072 Human

Unigene: 477783 Mouse

Unigene: 201860 Rat



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

该基因编码可能参与钴胺素转运和代谢的溶酶体膜蛋白。该蛋白还与大型肝炎δ抗原相互作用,并可能是肝炎δ病毒的核质穿梭所必需的。该基因的突变与维生素B12代谢紊乱有关,这种代谢紊乱称为同型半胱氨酸尿症-巨幼细胞性贫血互补型F。[由RefSeq提供,2009年10月]