MCCC1 Antikörper (AA 104-715)

Produktnummer ABIN7599657

Dieser Anti-MCCC1 Antikörper ist ein Kaninchen Polyklonal Antikörper zur Detektion von MCCC1 in WB, ELISA, IHC, ICC und IF. Geeignet für Human, Maus und Ratte.

Kurzübersicht für MCCC1 Antikörper (AA 104-715) (ABIN7599657)

Target: MCCC1 (Methylcrotonoyl-CoA Carboxylase 1 (Alpha) (MCCC1))

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser MCCC1 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), ELISA, Immunohistochemistry (IHC), Immunocytochemistry (ICC), Immunofluorescence (IF)

Produktdetails anti-MCCC1 Antikörper

Bindungsspezifität: AA 104-715

Verwendungszweck: Anti-MCCC1 Antibody Picoband®

Kreuzreaktivität (Details): No cross-reactivity with other proteins.

Produktmerkmale: Anti-MCCC1 Antibody Picoband® (ABIN7599657). Tested in ELISA, IF, IHC, ICC, WB applications. This antibody reacts with Human, Mouse, Rat. The brand Picoband indicates this is a premium antibody that guarantees superior quality, high affinity, and strong signals with minimal background in Western blot applications. Only our best-performing antibodies are designated as Picoband, ensuring unmatched performance.

Aufreinigung: Immunogen affinity purified.

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

Isotyp: IgG

Anwendungsinformationen

Applikationshinweise: Western blot, 0.25-0.5 μg/mL, Human, Mouse, Rat
Immunohistochemistry, 2-5 μg/mL, Human
Immunocytochemistry/Immunofluorescence, 5 μg/mL, Human
ELISA, 0.1-0.5 μg/mL, -
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.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Lyophilized

Rekonstitution: Adding 0.2 mL of distilled water will yield a concentration of 500 μg/mL.

Konzentration: 500 μg/mL

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

Lagerung: 4 °C,-20 °C

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

Details zu MCCC1

Target: MCCC1 (Methylcrotonoyl-CoA Carboxylase 1 (Alpha) (MCCC1))

Andere Bezeichnung: MCCC1

Hintergrund:

Synonyms: BAG family molecular chaperone regulator 5,BAG-5,Bcl-2-associated athanogene 5,BAG5,KIAA0873,

Tissue Specificity: Expressed in all tissues examined with lower levels in brain and testis.

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.

Molekulargewicht: 80 kDa

Gen-ID: 56922