MCCC2 Antibody

說明書

產品詳情

Uniprot No.：

Q9HCC0
基因名：

MCCC2
別名：

MCCC2 antibody; MCCBMethylcrotonoyl-CoA carboxylase beta chain antibody; mitochondrial antibody; MCCase subunit beta antibody; EC 6.4.1.4 antibody; 3-methylcrotonyl-CoA carboxylase 2 antibody; 3-methylcrotonyl-CoA carboxylase non-biotin-containing subunit antibody; 3-methylcrotonyl-CoA:carbon dioxide ligase subunit beta antibody
宿主：

Rabbit
反應種屬：

Human,Mouse,Rat
免疫原：

Human MCCC2
免疫原種屬：

Homo sapiens (Human)
抗體亞型：

IgG
純化方式：

Antigen Affinity Purified
濃度：

It differs from different batches. Please contact us to confirm it.
保存緩沖液：

PBS with 0.1% Sodium Azide, 50% Glycerol, pH 7.3. -20°C, Avoid freeze / thaw cycles.
產品提供形式：

Liquid
應用范圍：

ELISA,WB,IHC
Protocols：

ELISA Protocol
Western Blotting (WB) Protocol
Immunohistochemistry (IHC) Protocol
儲存條件：

Upon receipt, store at -20°C or -80°C. Avoid repeated freeze.
貨期：

Basically, we can dispatch the products out in 1-3 working days after receiving your orders. Delivery time maybe differs from different purchasing way or location, please kindly consult your local distributors for specific delivery time.
用途：

For Research Use Only. Not for use in diagnostic or therapeutic procedures.

功能：

Carboxyltransferase 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.
基因功能參考文獻：
1. Novel mutation in MCCC2 gene was identified in Chinese population. PMID: 25382614
2. Mutation in 3-methylcrotonyl CoA carboxylase 2 gene is associated with 3-methylcrotonyl-CoA carboxylase deficiency. PMID: 22264772
3. The amino-termini containing 20 amino acids (MCCbeta) were both necessary and sufficient for targeting. Structural requirements for mitochondrial import were defined by site-directed mutagenesis. PMID: 16023992
4. The Kd value of soraphen A for the BC domains of human ACC1 and ACC2 is 1 nM. This high binding affinity is mainly due to the extensive interactions between soraphen A and the human biotin carboxylase domain PMID: 17876819
相關疾病：

3-methylcrotonoyl-CoA carboxylase 2 deficiency (MCC2D)
亞細胞定位：

Mitochondrion matrix.
蛋白家族：

AccD/PCCB family
數據庫鏈接：

HGNC: 6937

OMIM: 210210

KEGG: hsa:64087

STRING: 9606.ENSP00000343657

UniGene: Hs.604789

YWHAG Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
YWHAB Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC, IF, FC

Species Reactivity: Human, Mouse, Rat
XRCC5 Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC, IF

Species Reactivity: Human
XPNPEP1 Recombinant Monoclonal Antibody

Applications: ELISA, IHC, FC

Species Reactivity: Human
XDH Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
WT1 Recombinant Monoclonal Antibody

Applications: ELISA, WB, IHC

Species Reactivity: Human
WDR5 Recombinant Monoclonal Antibody

Applications: ELISA, IHC, FC

Species Reactivity: Human
VIM Recombinant Monoclonal Antibody

Applications: ELISA, IHC, IF

Species Reactivity: Human