Storage instructionsShipped at 4°C. Upon delivery aliquot and store at -20°C. Avoid freeze / thaw cycles.
Storage bufferConstituent: PBS
Concentration information loading...
PurityImmunogen affinity purified
Our Abpromise guarantee covers the use of ab16307 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IHC-P||Use at an assay dependent dilution. PubMed: 19699272|
|WB||1/2000. Predicted molecular weight: 78 kDa.|
PathwayMetabolic intermediate metabolism; propanoyl-CoA degradation; succinyl-CoA from propanoyl-CoA: step 1/3.
Involvement in diseaseDefects in PCCA are the cause of propionic acidemia type I (PA-1) [MIM:606054]. PA-1 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein.
Sequence similaritiesContains 1 ATP-grasp domain.
Contains 1 biotin carboxylation domain.
Contains 1 biotinyl-binding domain.
Cellular localizationMitochondrion matrix.
- Information by UniProt
- mitochondrial antibody
- Pcca antibody
- pccA COMPLEMENTATION GROUP antibody
This product has been referenced in:
- Ballhausen D et al. Evidence for catabolic pathway of propionate metabolism in CNS: expression pattern of methylmalonyl-CoA mutase and propionyl-CoA carboxylase alpha-subunit in developing and adult rat brain. Neuroscience 164:578-87 (2009). IHC-P ; Rat . Read more (PubMed: 19699272) »