Anti-MCCC2 Antibody (CAB15181)

Antibody Name:	Anti-MCCC2 Antibody
Antibody SKU:	CAB15181
Antibody Size:	20uL, 50uL, 100uL
Application:	WB IHC IF
Reactivity:	Human, Mouse, Rat
Host Species:	Rabbit
Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 284-563 of human MCCC2 (NP_071415.1).

Application:	WB IHC IF
Recommended Dilution:	WB 1:500 - 1:2000 IHC 1:50 - 1:100 IF 1:50 - 1:100
Reactivity:	Human, Mouse, Rat
Positive Samples:	22Rv1, HeLa, HepG2, U-251MG, Mouse liver, Mouse kidney

Immunogen:	Recombinant fusion protein containing a sequence corresponding to amino acids 284-563 of human MCCC2 (NP_071415.1).
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:	LHLT RKVV RNLN YQKK LDVT IEPS EEPL FPAD ELYG IVGA NLKR SFDV REVI ARIV DGSR FTEF KAFY GDTL VTGF ARIF GYPV GIVG NNGV LFSE SAKK GTHF VQLC CQRN IPLL FLQN ITGF MVGR EYEA EGIA KDGA KMVA AVAC AQVP KITL IIGG SYGA GNYG MCGR AYSP RFLY IWPN ARIS VMGG EQAA NVLA TITK DQRA REGK QFSS ADEA ALKE PIIK KFEE EGNP YYSS ARVW DDGI IDPA DTRL VLGL SFSA ALNA PIEK TDFG IFRM
Gene ID:	64087
Uniprot:	Q9HCC0
Cellular Location:	Mitochondrion matrix
Calculated MW:	57kDa/61kDa
Observed MW:	61kDa

Synonyms:	MCCC2, MCCB
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.

UniProt Protein Function:	MCCC2: Defects in MCCC2 are the cause of methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D). 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. Belongs to the AccD/PCCB family. 2 isoforms of the human protein are produced by alternative splicing.
UniProt Protein Details:	Protein type:Mitochondrial; Ligase; Amino Acid Metabolism - valine, leucine and isoleucine degradation; EC 6.4.1.4 Chromosomal Location of Human Ortholog: 5q12-q13 Cellular Component: cytosol; mitochondrial matrix; mitochondrion Molecular Function: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 2 Deficiency
NCBI Summary:	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]
UniProt Code:	Q9HCC0
NCBI GenInfo Identifier:	20138731
NCBI Gene ID:	64087
NCBI Accession:	Q9HCC0.1
UniProt Secondary Accession:	Q9HCC0,Q96C27, Q9Y4L7, A6NIY9,
UniProt Related Accession:	Q9HCC0
Molecular Weight:	57,519 Da
NCBI Full Name:	Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
NCBI Synonym Full Names:	methylcrotonoyl-CoA carboxylase 2
NCBI Official Symbol:	MCCC2
NCBI Official Synonym Symbols:	MCCB
NCBI Protein Information:	methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
UniProt Protein Name:	Methylcrotonoyl-CoA carboxylase beta chain, mitochondrial
UniProt Synonym Protein Names:	3-methylcrotonyl-CoA carboxylase 2; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta
Protein Family:	Methylcrotonoyl-CoA carboxylase
UniProt Gene Name:	MCCC2
UniProt Entry Name:	MCCB_HUMAN

	Western blot analysis of extracts of various cell lines, using MCCC2 Rabbit pAb (CAB15181) 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.
	Immunofluorescence analysis of U2OS cells using MCCC2 antibody (CAB15181) at dilution of 1:100. Blue: DAPI for nuclear staining.

Antibodies

Anti-MCCC2 Antibody (CAB7990)

MCCC2 Antibody (PACO10414)

Secondary Antibody

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