Name: Anti-MCCC1 Antibody (CAB10020)
Brand: Cell Biology Antibodies 1
SKU: CAB10020
Availability: OutOfStock

Antibody Name:	Anti-MCCC1 Antibody
Antibody SKU:	CAB10020
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC
Reactivity:	Human, Mouse
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 526-725 of human MCCC1 (NP_064551.3).

Application:	WB IHC
Recommended Dilution:	WB 1:500 - 1:1000 IHC 1:50 - 1:100
Reactivity:	Human, Mouse
Positive Samples:	Jurkat, Mouse kidney

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 526-725 of human MCCC1 (NP_064551.3).
Purification Method:	Affinity purification
Storage Buffer:	Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:	IgG
Sequence:	FTLQ AHDQ FSPF SSSS GRRL NISY TRNM TLKD GKNN VAIA VTYN HDGS YSMQ IEDK TFQV LGNL YSEG DCTY LKCS VNGV ASKA KLII LENT IYLF SKEG SIEI DIPV PKYL SSVS SQET QGGP LAPM TGTI EKVF VKAG DKVK AGDS LMVM IAMK MEHT IKSP KDGT VKKV FYRE GAQA NRHT PLVE FEEE ESDK RESE
Gene ID:	56922
Uniprot:	Q96RQ3
Cellular Location:	Mitochondrion matrix
Calculated MW:	80kDa
Observed MW:	80kDa

Synonyms:	MCCC1, MCC-B, MCCA
Background:	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.

UniProt Protein Function:	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
UniProt Protein Details:	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.
NCBI Summary:	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 Code:	Q96RQ3
NCBI GenInfo Identifier:	116805327
NCBI Gene ID:	56922
NCBI Accession:	NP_064551.3
UniProt Secondary Accession:	Q96RQ3,Q99MR8,
UniProt Related Accession:	Q96RQ3
Molecular Weight:	80,473 Da
NCBI Full Name:	methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
NCBI 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 carbox
UniProt Protein Name:	Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
UniProt Synonym Protein Names:	3-methylcrotonyl-CoA carboxylase 1; 3-methylcrotonyl-CoA carboxylase biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha
Protein Family:	Microcin
UniProt Gene Name:	MCCC1
UniProt Entry Name:	MCCA_HUMAN

Western blot analysis of extracts of various cell lines, using MCCC1 antibody (CAB10020) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.

Antibodies

MCCC1 Antibody (PACO10413)

Secondary Antibody

Anti-HRP Goat Anti-Rabbit IgG (H+L) Antibody (CABS014)