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

MCCC1 (MCCA, Methylcrotonoyl-CoA Carboxylase Subunit alpha, Mitochondrial, 3-methylcrotonyl-CoA Carboxylase 1, 3-methylcrotonyl-CoA Carboxylase Biotin-containing Subunit, 3-methylcrotonyl-CoA:carbon Dioxide Ligase Subunit alpha, DKFZp686B20267, FLJ25545,

Cat. Num. AAA646691

• Gene Names: MCCC1; MCCA; MCC-B
• Reactivity: Human
• Applications: Western Blot
• Purity: Affinity Purified; Purified by Protein A affinity chromatography.
• Synonyms: MCCC1; Polyclonal Antibody; MCCC1 (MCCA; Methylcrotonoyl-CoA Carboxylase Subunit alpha; Mitochondrial; 3-methylcrotonyl-CoA Carboxylase 1; 3-methylcrotonyl-CoA Carboxylase Biotin-containing Subunit; 3-methylcrotonyl-CoA:carbon Dioxide Ligase Subunit alpha; DKFZp686B20267; FLJ25545; ; Anti -MCCC1 (MCCA; anti-MCCC1 antibody

• Host: Mouse
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human MCCC1.
• Purity/Purification: Affinity Purified; Purified by Protein A affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2.
• Sequence: MAAASAVSVLLVAAERNRWHRLPSLLLPPRTWVWRQRTMKYTTATGRNITKVLIANRGEIACRVMRTAKKLGVQTVAVYSEADRNSMHVDMADEAYSIGPAPSQQSYLSMEKIIQVAKTSAAQAIHPGCGFLSENMEFAELCKQEGIIFIGPPPSAIRDMGIKSTSKSIMAAAGVPVVEGYHGEDQSDQCLKEHARRIGYPVMIKAVRGGGGKGMRIVRSEQEFQEQLESARREAKKSFNDDAMLIEKFVDTPRHVEVQVFGDHHGNAVYLFERDCSVQRRHQKIIEEAPAPGIKSEVRKKLGEAAVRAAKAVNYVGAGTVEFIMDSKHNFCFMEMNTRLQVEHPVTEMITGTDLVEWQLRIAAGEKIPLSQEEITLQGHAFEARIYAEDPSNNFMPVAGPLVHLSTPRADPSTRIETGVRQGDEVSVHYDPMIAKLVVWAADRQAALTKLRYSLRQYNIVGLPTNIDFLLNLSGHPEFEAGNVHTDFIPQHHKQLLLSRKAAAKESLCQAALGLILKEKAMTDTFTLQAHDQFSPFSSSSGRRLNISYTRNMTLKDGKNNVAIAVTYNHDGSYSMQIEDKTFQVLGNLYSEGDCTYLKCSVNGVASKAKLIILENTIYLFSKEGSIEIDIPVPKYLSSVSSQETQGGPLAPMTGTIEKVFVKAGDKVKAGDSLMVMIAMKMEHTIKSPKDGTVKKVFYREGAQANRHTPLVEFEEEESDKRESE

• Applicable Applications for anti-MCCC1 antibody: Western Blot (WB)
• Application Notes: Suitable for use in Western Blot.
• Immunogen: Full length human MCCC1, aa1-725 (AAH04214.1).
• Preparation and Storage: May be stored at 4 degree C for short-term only. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. Aliquots are stable for at least 12 months. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western Blot analysis of MCCC1 expression in transfected 293T cell line by MCCC1 polyclonal antibody. Lane 1: MCCC1 transfected lysate (79.75kD). Lane 2: Non-transfected lysate.)

Related Product Information for anti-MCCC1 antibody

MCCC1 is 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.

Product Categories/Family for anti-MCCC1 antibody

Antibodies; Abs to Enzymes

NCBI and Uniprot Product Information

• NCBI GI #: 116805327
• NCBI GeneID: 56922
• NCBI Accession #: NP_064551.3
• NCBI GenBank Nucleotide #: NM_020166.3
• UniProt Accession #: Q96RQ3; Q59ES4; Q9H959; Q9NS97
• Molecular Weight: 80,473 Da
• NCBI Official Full Name: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
• NCBI Official Synonym Full Names: methylcrotonoyl-CoA carboxylase 1 (alpha)
• NCBI Official Symbol: MCCC1
• NCBI Official Synonym Symbols: MCCA; MCC-B
• NCBI Protein Information: methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial; MCCase subunit alpha; 3-methylcrotonyl-CoA carboxylase 1; methylcrotonoyl-Coenzyme A carboxylase 1 (alpha); 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit
• 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.