Product Name	MCCC2
Chinese Name	MCCC2蛋白抗体
Alias	3 methylcrotonyl CoA carboxylase 2; 3 methylcrotonyl CoA carboxylase non biotin containing subunit; 3 methylcrotonyl CoA:carbon dioxide ligase subunit beta; 3-methylcrotonyl-CoA carboxylase 2; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta; Biotin carboxylase; MCCase subunit beta; MCCB; MCCB_HUMAN; MCCC 2; Mccc2; Methylcrotonoyl CoA carboxylase 2 (beta); Methylcrotonoyl CoA carboxylase beta chain mitochondrial; Methylcrotonoyl Coenzyme A carboxylase 2 (beta); Methylcrotonoyl-CoA carboxylase beta chain; mitochondrial; Non biotin containing subunit of 3 methylcrotonyl CoA carboxylase.
Research Area	Cell biology  Signal transduction  The new supersedes the old
Immunogen Species	Rabbit
Clonality	Polyclonal
React Species	Human, Mouse,  (predicted: Rat, Dog, Pig, Cow, Horse, Rabbit, )
Applications	WB=1:500-2000  not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
Theoretical molecular weight	61kDa
Cellular localization	cytoplasmic
Form	Liquid
Concentration	1mg/ml
immunogen	KLH conjugated synthetic peptide derived from human MCCC2: 351-450/563
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 small subunit of 3-methylcrotonyl-CoA carboxylase. This enzyme functions as a heterodimer and catalyzes the carboxylation of 3-methylcrotonyl-CoA to form 3-methylglutaconyl-CoA. Mutations in this gene are associated with 3-Methylcrotonylglycinuria, an autosomal recessive disorder of leucine catabolism. [provided by RefSeq, Jul 2008] Function: Carboxyltransferase subunit of the 3-methylcrotonyl-CoA carboxylase, an enzyme that catalyzes the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a critical step for leucine and isovaleric acid catabolism. Subunit: Probably a dodecamer composed of six biotin-containing alpha subunits (MCCC1) and six beta (MCCC2) subunits. Subcellular Location: Mitochondrion matrix DISEASE: Defects in MCCC2 are the cause of methylcrotonoyl-CoA carboxylase deficiency type 2 (MCC2 deficiency) [MIM:210210]. MCC2 deficiency is an autosomal recessive disorder of leucine catabolism. The phenotype is variable, ranging from neonatal onset with severe neurological involvement to asymptomatic adults. There is a characteristic organic aciduria with massive excretion of 3-hydroxyisovaleric acid and 3-methylcrotonylglycine, usually in combination with a severe secondary carnitine deficiency. Similarity: Belongs to the AccD/PCCB family. Contains 1 carboxyltransferase domain. SWISS: Q9HCC0 Gene ID: 64087 Database links: Entrez Gene: 64087 Human Entrez Gene: 78038 Mouse Entrez Gene: 361884 Rat Omim: 609014 Human SwissProt: Q9HCC0 Human SwissProt: Q3ULD5 Mouse SwissProt: Q5XIT9 Rat Unigene: 604789 Human Unigene: 137327 Mouse Unigene: 33635 Rat
Product Picture	Sample: Kidney (Mouse) Lysate at 40 ug DU145(Human) Cell Lysate at 30 ug Primary: Anti-MCCC2 (SL18719R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 61 kD Observed band size: 61 kD

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