Anti-MCCC2 Antibody (CAB15181)
- SKU:
- CAB15181
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Frequently bought together:
Description
| 抗体名: | Anti-MCCC2 Antibody |
| 抗体コード: | CAB15181 |
| 抗体サイズ: | 20uL, 50uL, 100uL |
| 申し込み: | WB IHC IF |
| 反応性: | Human, Mouse, Rat |
| 宿主種: | Rabbit |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 284-563 of human MCCC2 (NP_071415.1). |
| 申し込み: | WB IHC IF |
| 推奨希釈: | WB 1:500 - 1:2000 IHC 1:50 - 1:100 IF 1:50 - 1:100 |
| 反応性: | Human, Mouse, Rat |
| ポジティブサンプル: | 22Rv1, HeLa, HepG2, U-251MG, Mouse liver, Mouse kidney |
| 免疫原: | Recombinant fusion protein containing a sequence corresponding to amino acids 284-563 of human MCCC2 (NP_071415.1). |
| 精製方法: | Affinity purification |
| ストレージバッファ: | Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
| アイソタイプ: | IgG |
| 順序: | 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 |
| 遺伝子ID: | 64087 |
| Uniprot: | Q9HCC0 |
| セルラーロケーション: | Mitochondrion matrix |
| 計算された分子量: | 57kDa/61kDa |
| 観察された分子量: | 61kDa |
| 同義語: | MCCC2, MCCB |
| バックグラウンド: | 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. |
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