产品货号 :  mlR18310

英文名称 :  LMBRD2

中文名称 :  LMBRD2蛋白抗体

别    名 :  LMBD2_HUMAN; LMBR1 domain-containing protein 2; LMBRD2.  

研究领域 :  细胞生物  免疫学  

抗体来源 :  Rabbit

克隆类型 :  Polyclonal

交叉反应 :  Human, Mouse, Rat, Horse, Rabbit,

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 :  81kDa

细胞定位 :  细胞膜

性    状 :  Lyophilized or Liquid

浓    度 :  1mg/ml

免 疫 原 :  KLH conjugated synthetic peptide derived from human LMBRD2:551-650/695 <Cytoplasmic>

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 :   Vitamin B12 (cobalamin) is essential in animals and humans for metabolism of methylmalonic acid, for the remethylation of homocysteine to methionine and, consequently, for all S-adenosylmethionine-dependent methylation reactions, including DNA synthesis. The lysosomal cobalamin transporter is required for the export cobalamin from lysosomes allowing its conversion to cofactors. Defects in LMBRD1 are the cause of methylmalonic aciduria and homocystinuria type cblF (MMAFHC), 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) due to accumulation of cobalamin in lysosomes. Clinical features of MMAFHC include developmental delay, stomatitis, glossitis, seizures and methylmalonic aciduria in response to vitamin B12. LMBRD2 (LMBR1 domain containing 2) is a 695 amino acid multi-membrane protein that may have similar functions as LMBR1.

Subcellular Location:

Membrane.

Similarity:

Belongs to the LIMR family.

SWISS:

Q68DH5

Gene ID:

92255

Important Note:

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