PCCA Mouse anti-Human, Clone: OTI1E9, Invitrogen
Mouse Monoclonal Antibody
Manufacturer: Invitrogen MA527386
DescriptionThis protein is a key enzyme in the catabolic pathway of odd-chain fatty acids, isoleucine, threonine, methionine, and valine. It is probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits. The protein is located in the mitochondrial matrix. Defects in PCCA are the cause o fpropionic acidemia type I (PA-1) PA-1 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, develop-mental retardation, and intolerance to protein.
|PBS with 1% BSA, 50% glycerol and 0.02% sodium azide; pH 7.3|
|PCCase alpha subunit, pccA complementation group, propanoyl-CoA:carbon dioxide ligase alpha subunit, propionyl Coenzyme A carboxylase, alpha polypeptide, propionyl-CoA carboxylase alpha chain, mitochondrial|
|-20° C, Avoid Freeze/Thaw Cycles|
|Immunohistochemistry (Paraffin), Western Blot|
|Full length human recombit protein of PCCA produced in HEK293T cell|
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