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Invitrogen
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For reconstitution, add sterile, distilled water to achieve a final antibody concentration of 1 mg/mL. Gently shake to solubilize the protein completely. Do not vortex. Reconstituted products should be stored at -80 °.
This antibody can also be cross referenced by the term FA19-1.
This 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) [MIM:606054]. 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.
仅用于科研。不用于诊断过程。未经明确授权不得转售。