MCCC1 Antibody (PACO45560)
- SKU:
- PACO45560
- Product type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Application:
- IP
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Description
抗体名: | MCCC1 Antibody (PACO45560) |
抗体コード: | PACO45560 |
サイズ: | 50ul |
宿主種: | Rabbit |
申し込み: | ELISA, IHC, IP |
推奨される希釈: | ELISA:1:2000-1:10000, IHC:1:20-1:200, IP:1:200-1:2000 |
反応性: | Human |
免疫原: | Recombinant Human Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial protein (526-725AA) |
憲法: | Liquid |
ストレージバッファ: | PBS with 0.02% sodium azide, 50% glycerol, pH7.3. |
精製方法: | Antigen Affinity Purified |
抗体のクローン性: | Polyclonal |
アイソタイプ: | IgG |
Conjugate: | Non-conjugated |
![]() | Immunohistochemistry of paraffin-embedded human liver cancer using PACO45560 at dilution of 1:100. |
![]() | Immunoprecipitating MCCC1 in HEK293 whole cell lysate. Lane 1: Rabbit control IgG instead of PACO45560 in HEK293 whole cell lysate.For western blotting, a HRP-conjugated Protein G antibody was used as the secondary antibody (1/2000). Lane 2: PACO45560 (8µg) + HEK293 whole cell lysate (500µg). Lane 3: HEK293 whole cell lysate (10µg). |
![]() | Immunohistochemistry of paraffin-embedded human colon cancer using PACO45560 at dilution of 1:100. |
バックグラウンド: | 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. |
シノニム: | Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial (MCCase subunit alpha) (EC 6.4.1.4) (3-methylcrotonyl-CoA carboxylase 1) (3-methylcrotonyl-CoA carboxylase biotin-containing subunit) (3-methylcrotonyl-CoA:carbon dioxide ligase subunit alpha), MCCC1, MCCA |
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 |