Product Name	MUT/Methylmalonyl Coenzyme A mutase
Chinese Name	甲基丙二酰异构酶抗体
Alias	MCM; Methylmalonyl CoA isomerase; Methylmalonyl CoA mutase mitochondrial; Methylmalonyl Coenzyme A mutase; Methylmalonyl-CoA isomerase; Methylmalonyl-CoA mutase; mitochondrial; Mut; MUTA_HUMAN.
Research Area	Cell biology  Signal transduction
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	(predicted: Human, Mouse, Rat, )
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	79kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human MUT/Methylmalonyl Coenzyme A mutase: 451-550/750
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	This gene encodes the mitochondrial enzyme methylmalonyl Coenzyme A mutase. In humans, the product of this gene is a vitamin B12-dependent enzyme which catalyzes the isomerization of methylmalonyl-CoA to succinyl-CoA, while in other species this enzyme may have different functions. Mutations in this gene may lead to various types of methylmalonic aciduria. [provided by RefSeq, Jul 2008] Function: Involved in the degradation of several amino acids, odd-chain fatty acids and cholesterol via propionyl-CoA to the tricarboxylic acid cycle. MCM has different functions in other species. Subcellular Location: Mitochondrion matrix. DISEASE: Defects in MUT are the cause of methylmalonic aciduria type mut (MMAM) [MIM:251000]. MMAM is an often fatal disorder of organic acid metabolism. Common clinical features include lethargy, vomiting, failure to thrive, hypotonia, neurological deficit and early death. Two forms of the disease are distinguished by the presence (mut-) or absence (mut0) of residual enzyme activity. Mut0 patients have more severe neurological manifestations of the disease than do MUT- patients. MMAM is unresponsive to vitamin B12 therapy. Similarity: Belongs to the methylmalonyl-CoA mutase family. Contains 1 B12-binding domain. SWISS: P22033 Gene ID: 4594 Database links: Entrez Gene: 4594 Human Entrez Gene: 422049 Chicken Entrez Gene: 17850 Mouse Entrez Gene: 688517 Rat Entrez Gene: 569581 Zebrafish Omim: 609058 Human SwissProt: P22033 Human SwissProt: P16332 Mouse Unigene: 485527 Human Unigene: 259884 Mouse

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