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MCCB rabbit pAb

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],

Product Specifications

Background

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]

Product Name Alternative

Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCase subunit beta) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 2) (3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta)

UniProt

Q9HCC0

Swiss Prot

Q9HCC0

Reactivity

Human; Mouse; Rat

Immunogen

Synthesized peptide derived from human MCCB AA range: 204-254

Target

MCCB

Clonality

Polyclonal

Source

Rabbit

Applications

WB

Concentration

1 mg/ml

Dilution

WB 1:500-2000

Buffer

-20°C/1 year

Molecular Weight

60kD

Storage Conditions

-20°C/1 year

Observed Molecular Weight

60kD

Fragment

IgG

Subcellular Location

Mitochondrion matrix .

Other Product Names

Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial (MCCase subunit beta) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 2) (3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta)

Gene ID (Human)

64087

Available Sizes

Frequently Asked Questions

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