Anti-MCCC1 Antibody Picoband®

SKU: A07441-1

Size: 100 μg

Storage: At -20℃ for one year from date of receipt. After reconstitution, at 4℃ for one month. It can also be aliquotted and stored frozen at -20℃ for six months. Avoid repeated freezing and thawing.

Form: Lyophilized

Reactivity: Human,Mouse,Rat

Applications: WB,IHC,ICC,IF,ELISA

Application Details: Western blot, 0.25-0.5 μg/ml, Human, Mouse, Rat
Immunohistochemistry (Paraffin-embedded Section), 2-5 μg/ml, Human
Immunocytochemistry/Immunofluorescence, 5 μg/ml, Human
ELISA, 0.1-0.5 μg/ml, -

Gene Name: MCCC1

Specificity:

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.

Immunogen: E.coli-derived human MCCC1 recombinant protein (Position: Q104-E715).

Clonality: Polyclonal

Contents: Each vial contains 4 mg Trehalose, 0.9 mg NaCl, 0.2 mg Na2HPO4.

Purification: Immunogen affinity purified.

Reconstitution: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Reference: 1. Baumgartner, M. R., Almashanu, S., Suormala, T., Obie, C., Cole, R. N., Packman, S., Baumgartner, E. R., Valle, D. The molecular basis of human 3-methylcrotonyl-CoA carboxylase deficiency. J. Clin. Invest. 107: 495-504, 2001. 2. Baumgartner, M. R., Dantas, M. F., Suormala, T., Almashanu, S., Giunta, C., Friebel, D., Gebhardt, B., Fowler, B., Hoffmann, G. F., Baumgartner, E. R., Valle, D. Isolated 3-methylcrotonyl-CoA carboxylase deficiency: evidence for an allele-specific dominant negative effect and responsiveness to biotin therapy. Am. J. Hum. Genet. 75: 790-800, 2004. 3. Gallardo, M. E., Desviat, L. R., Rodriguez, J. M., Esparza-Gordillo, J., Perez-Cerda, C., Perez, B., Rodriguez-Pombo, P., Criado, O., Sanz, R., Morton, D. H., Gibson, K. M., Le, T. P., Ribes, A., Rodriguez de Cordoba, S., Ugarte, M., Penalva, M. A. The molecular basis of 3-methylcrotonylglycinuria, a disorder of leucine catabolism. Am. J. Hum. Genet. 68: 334-346, 2001.

Uniprot ID: Q96RQ3

Host: Rabbit

Concentration: Adding 0.2 ml of distilled water will yield a concentration of 500 μg/ml.

Conjugate:

Cross Reactivity: No cross-reactivity with other proteins.

Isotype: Rabbit IgG

Phospho_site:

Clone Number:

Observed Molecular Weight: 80 kDa

Calculated Molecular Weight: 80 kDa

Gene ID: 56922

Protein Name: Methylcrotonoyl-CoA carboxylase subunit alpha, mitochondrial

Gene Full Name: methylcrotonyl-CoA carboxylase subunit 1

Synonyms: MCC B; MCCA; MCCase subunit alpha; MCCC1