MCEE蛋白抗体
规格：1mg/1ml
英文名： MCEE
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; methylmalo
分子量： 19kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human MCEE
交叉反应：Human, Mouse,
细胞定位：
MCEE蛋白抗体产品介绍：background: 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 aciduriaMCEE蛋白抗体 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,MCEE蛋白抗体 pancytopenia and coma. Similarity: Belongs to the glyoxalase I family. Database links: Entrez Gene: 84693 Human Entrez Gene: 73724 Mouse Omim: 608419 Human SwissProt: Q96PE7 Human SwissProt: Q9D1I5 Mouse Unigene: 94949 Human Unigene: 10093 Mouse Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
MCEE蛋白抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 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.
研究领域：细胞生物 **学
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid