Chinese (Simplified)
English
German
Korean
Spanish
Sign up for an account to enjoy easy online shopping and instant order tracking.
| Cat.No | ACP12856 | Target Name | MCCC1 |
|---|---|---|---|
| Form | Lyophilized powder | Expression System | Custom Production. Please inquire and provide the desire expression system. |
| Expression Range | 42-725 | Protein Length | Full Length of Mature Protein |
| Purity | >85% (SDS-PAGE) | Storage Buffer | 5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0. |
| Target Species | Human | 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.
Free shipping on orders $999+
Advenit post multos Scudilo Scutariorum tribunus velament
We accept credit cards, PayPal, and bank wires
Responsive and knowledgable customer service via email, text or phone
Notification
