产品货号 :  mlR18720

英文名称 :  MCEE

中文名称 :  MCEE蛋白抗体

别    名 :  DL methylmalonyl CoA racemase; DL-methylmalonyl-CoA racemase; EC 5.1.99.1; GLOD2; Glyoxalase domain containing 2; MCEE; MCEE_HUMAN; Methylmalonyl CoA epimerase; Methylmalonyl-CoA epimerase; methylmalonyl-CoA epimerase, mitochondrial; mitochondrial; OTTHUMP00000160122.  

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, Chicken, Dog, Horse,

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 :  15kDa

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 MCEE:101-176/176

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 :   MCEE is a 176 amino acid mitochondrial protein that belongs to the glyoxalase I family. MCEE catalyzes the interconversion of D- and L-methylmalonyl-CoA during the breakdown of branched chain amino acids. The gene encoding MCEE maps to human chromosome 2p13.3. Defects in the MCEE gene are the cause of an autosomal recessive disease known as methylmalonyl-CoA epimerase deficiency (MCEE deficiency), methylmalonyl-CoA racemase deficiency or methylmalonic aciduria type 3, which is characterized by mild to moderate methylmalonic aciduria.

Subcellular Location:

Mitochondrion.

DISEASE:

Defects in MCEE are a cause of methylmalonyl-CoA epimerase deficiency (MCEE deficiency) [MIM:251120]; also known as methylmalonyl-CoA racemase deficiency or methylmalonic aciduria type 3. MCEE deficiency is an autosomal recessive inborn error of amino acid metabolism, involving valine, threonine, isoleucine and methionine. This organic aciduria may present in the neonatal period with life-threatening metabolic acidosis, hyperammonemia, feeding difficulties, pancytopenia and coma.

Similarity:

Belongs to the glyoxalase I family.

SWISS:

Q96PE7

Gene ID:

84693

Important Note:

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