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The antibody against MCCC1 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 640-725 of human MCCC1 (NP_064551.3) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IHC-P, ELISA.
The antibody against MCCC1 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 640-725 of human MCCC1 (NP_064551.3) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IHC-P, ELISA.
| Cat.No | ADA-01191A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | MCCC1 |
| Target Synonyms | MCCA; MCC-B; MCCCalpha; MCCC1 | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.02% sodium azide, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | T-47D | Application | ELISA, WB, IHC-P |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 640-725 of human MCCC1 (NP_064551.3). | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | VSSQETQGGPLAPMTGTIEKVFVKAGDKVKAGDSLMVMIAMKMEHTIKSPKDGTVKKVFYREGAQANRHTPLVEFEEEESDKRESE | Uniprot ID | Q96RQ3 |
Uniprot Id
Q96RQ3
Target Species
Human
Target Name
MCCC1
Target Full Name
Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial
Target 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.
Target Involvement
3-methylcrotonoyl-CoA carboxylase 1 deficiency (MCC1D)
Target Subcellular Location
Mitochondrion matrix.
Target Synonyms
MCCC1; MCCAMethylcrotonoyl-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
Target 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.
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