Anti-MCCC1 Antibody (CAB10020)
- SKU:
- CAB10020
- Product type:
- Antibody
- Reactivity:
- Human
- Mouse
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Polyclonal Antibody
- Research Area:
- Cell Biology
Description
| 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. |
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