Product Name	MCEE
Chinese Name	MCEE蛋白抗体
Alias	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.
Research Area	Cell biology  immunology
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, Chicken, Dog, Horse, )
Applications	ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （Paraffin sections need antigen repair） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	15kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human MCEE: 101-176/176
Lsotype	IgG
Purification	affinity purified by Protein A
Buffer Solution	0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage	Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
Attention	This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed	PubMed
Product Detail	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 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

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