Rabbit MCCC1 Polyclonal Antibody | anti-MCCC1 antibody

MCCC1 Rabbit Polyclonal

Cat. Num. AAA7601280

• Gene Names: MCCC1; MCCA; MCC-B
• Reactivity: Human, Mouse, Rat
• Applications: ELISA, Western Blot, Immunohistochemistry
• Purity: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Synonyms: MCCC1; Polyclonal Antibody; MCCC1 Rabbit Polyclonal; anti-MCCC1 antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: >=95% as determined by SDS-PAGE; Immunogen Affinity Purified
• Form/Format: Liquid
• Sequence Length: 725

• Applicable Applications for anti-MCCC1 antibody: ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)
• Immunogen: Methylcrotonoyl-Coenzyme A carboxylase 1 (alpha)
• Preparation and Storage: PBS with 0.02% sodium azide and 50% glycerol pH 7.3, -20 degree C for 24 months (Avoid repeated freeze / thaw cycles.)

Immunohistochemistry (IHC)

(Immunohistochemistry of paraffin-embedded human liver cancer using MBS7601280 (MCCC1 antibody) at dilution of 1:100)

Western Blot (WB)

(mouse liver tissue were subjected to SDS PAGE followed by western blot with MBS7601280 (MCCC1 antibody) at dilution of 1:1200)

NCBI and Uniprot Product Information

• NCBI GI #: 116805327
• NCBI GeneID: 56922
• NCBI Accession #: NP_064551.3
• NCBI GenBank Nucleotide #: NM_020166.4
• Molecular Weight: 80,473 Da
• NCBI Official Full Name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 1
• NCBI Official Synonym Full Names: methylcrotonoyl-CoA carboxylase 1
• NCBI Official Symbol: MCCC1
• NCBI Official Synonym Symbols: MCCA; MCC-B
• NCBI Protein Information: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
• UniProt Protein Name: Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
• UniProt Gene Name: MCCC1
• UniProt Synonym Gene Names: MCCA; MCCase subunit alpha
• UniProt Entry Name: MCCA_HUMAN

NCBI Description

This gene encodes the large 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]

Uniprot Description

MCCC1: Defects in MCCC1 are the cause of methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D). 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.

Protein type: Ligase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 6.4.1.4; Mitochondrial

Chromosomal Location of Human Ortholog: 3q27

Cellular Component: cytosol; mitochondrial inner membrane; mitochondrial matrix; mitochondrion

Molecular Function: ATP binding; biotin binding; biotin carboxylase activity; metal ion binding; methylcrotonoyl-CoA carboxylase activity; protein binding

Biological Process: biotin metabolic process; branched chain family amino acid catabolic process; leucine catabolic process

Disease: 3-methylcrotonyl-coa Carboxylase 1 Deficiency