The MCCC1 Polyclonal Antibody (CAB10020) is a valuable tool for researchers studying MCCC1, a key enzyme involved in the mitochondrial pathway of propionate metabolism. This antibody, produced in rabbits, exhibits high reactivity with human samples and has been validated for use in Western blot applications. By specifically binding to the MCCC1 protein, this antibody allows for precise detection and analysis in a variety of cell types, making it an essential component for studies in metabolism and related disorders.MCCC1 is responsible for the conversion of 3-methylcrotonyl-CoA to 3-methylglutaconyl-CoA, a crucial step in the breakdown of certain amino acids and fatty acids for energy production.
Dysregulation of MCCC1 has been linked to metabolic disorders such as propionic acidemia, a rare genetic condition characterized by a buildup of toxic compounds in the body. Research into the function and regulation of MCCC1 is essential for understanding the pathophysiology of these conditions and developing targeted therapies for affected individuals.
Product Name:
MCCC1 Rabbit Polyclonal Antibody
SKU:
CAB10020
Size:
20uL, 100uL
Isotype:
IgG
Host Species:
Rabbit
Reactivity:
Human,Mouse,Rat
Immunogen:
Recombinant fusion protein containing a sequence corresponding to amino acids 640-725 of human MCCC1 (NP_064551.3).
Sequence:
VSSQ ETQG GPLA PMTG TIEK VFVK AGDK VKAG DSLM VMIA MKME HTIK SPKD GTVK KVFY REGA QANR HTPL VEFE EEES DKRE SE
Tested Applications:
WBIHC-PELISA
Recommended Dilution:
WB,1:1000 - 1:5000 IHC-P,1:50 - 1:100
Synonyms:
MCCA; MCC-B; MCCCalpha; MCCC1
Positive Sample:
T-47D
Conjugate:
Unconjugated
Cellular Localization:
Mitochondrion matrix.
Calculated MW:
80kDa
Observed MW:
726aa/80kDa
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.
Purification Method:
Affinity purification
Gene ID:
56922
Storage Buffer:
Store at -20℃. Avoid freeze / thaw cycles.Buffer: PBS with 0.02% sodium azide,50% glycerol,pH7.3.
Western blot analysis of lysates from T-47D cells, using MCCC1 Rabbit pAb (CAB10020) at 1:2000 dilution.Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution.Lysates/proteins: 25μg per lane.Blocking buffer: 3% nonfat dry milk in TBST.Detection: ECL Basic Kit (AbGn00020).Exposure time: 60s.
