Rabbit anti-Human MCCC1 Polyclonal Antibody | anti-MCCC1 antibody

MCCC1 Antibody - middle region

Cat. Num. AAA3221465

• Gene Names: MCCC1; MCCA; MCC-B
• Reactivity: Human
• Applications: Western Blot
• Purity: Affinity purified
• Synonyms: MCCC1; Polyclonal Antibody; MCCC1 Antibody - middle region; anti-MCCC1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Purity/Purification: Affinity purified
• Form/Format: Liquid. Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
• Sequence: Synthetic peptide located within the following region: SGRRLNISYTRNMTLKDGKNNVAIAVTYNHDGSYSMQIEDKTFQVLGNLY
• Sequence Length: 725

• Applicable Applications for anti-MCCC1 antibody: Western Blot (WB)
• Immunogen: The immunogen is a synthetic peptide directed towards the middle region of human MCCC1
• Preparation and Storage: For short term use, store at 2-8 degree C up to 1 week. For long term storage, store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Western Blot (WB)

(Host: RabbitTarget Name: MCCC1Sample Tissue: Human HeLa Whole Cell lysatesAntibody Dilution: 1ug/ml)

Related Product Information for anti-MCCC1 antibody

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.

Product Categories/Family for anti-MCCC1 antibody

Polyclonal; Cell Biology

NCBI and Uniprot Product Information

• NCBI GI #: 647569552
• NCBI GeneID: 56922
• NCBI Accession #: NP_001280202.1
• NCBI GenBank Nucleotide #: NM_001293273.1
• UniProt Accession #: Q96RQ3
• Molecular Weight: 80 kDa
• NCBI Official Full Name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial isoform 2
• 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

Function: Biotin-attachment 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. Ref.9

Catalytic activity: ATP + 3-methylcrotonoyl-CoA + HCO3- = ADP + phosphate + 3-methylglutaconyl-CoA. Ref.9

Cofactor: Biotin.

Pathway: Amino-acid degradation; L-leucine degradation; (S)-3-hydroxy-3-methylglutaryl-CoA from 3-isovaleryl-CoA: step 2/3.

Subunit structure: Probably a dodecamer composed of six biotin-containing alpha subunits (MCCC1) and six beta (MCCC2) subunits.

Subcellular location: Mitochondrion matrix Ref.8.

Involvement in disease: Methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D) [MIM:210200]: 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.1 Ref.3 Ref.4 Ref.12

Sequence similarities: Contains 1 ATP-grasp domain.Contains 1 biotin carboxylation domain.Contains 1 biotinyl-binding domain.

Sequence caution: The sequence BAD92974.1 differs from that shown. Reason: Erroneous initiation.