Search Thermo Fisher Scientific
Search Thermo Fisher Scientific
Recommended positive controls: HCT116.
Predicted reactivity: Mouse (84%), Rat (87%), Bovine (94%).
Store product as a concentrated solution. Centrifuge briefly prior to opening the vial.
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.
⚠WARNING: This product can expose you to chemicals including mercury, which is known to the State of California to cause birth defects or other reproductive harm. For more information go to www.P65Warnings.ca.gov.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: 1700055P21Rik; coagulation factor V/VIII-homology domains protein 1; CUB, LCCL and coagulation factor V/VIII-homology domains protein 1; discoidin; Discoidin, CUB and LCCL domain-containing protein 2; Endothelial and smooth muscle cell-derived neuropilin-like protein
Gene Aliases: CLCP1; DCBLD2; ESDN
UniProt ID: (Human) Q96PD2
Entrez Gene ID: (Human) 131566
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