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The antibody against MCCC1 was raised in Rabbit using the recombinant Protein corresponding to a sequence within amino acids 640-725 of human MCCC1(NP_064551.3) as the immunogen. The monoclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IF/ICC, IP, ELISA.
The antibody against MCCC1 was raised in Rabbit using the recombinant Protein corresponding to a sequence within amino acids 640-725 of human MCCC1(NP_064551.3) as the immunogen. The monoclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, IF/ICC, IP, ELISA.
Western blot analysis of various lysates using [KD Validated] MCCC1 Rabbit mAb at 1:3000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 45s.
| Cat.No | ADA-13319A | Clonality | Monoclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | MCCC1 |
| Target Synonyms | MCCA; MCC-B; MCCCalpha; [KD Validated] MCCC1 | Form | Liquid |
| Species Reactivity | Human, Mouse, Rat | Isotype | IgG |
| Storage Buffer | 50% Glycerol, 0.05% BSA, PBS with 0.05% proclin300, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | Rat heart, 293T, Mouse liver, U-2 OS | Application | ELISA, WB, IF/ICC, IP |
| Immunogen Description | Recombinant Protein corresponding to a sequence within 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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