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Human DCBLD2/ESDN quantitates human DCBLD2/ESDN in serum, plasma, supernatant. The assay will exclusively recognize both natural and recombinant human DCBLD2/ESDN.
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。
基因别名 : CLCP1, DCBLD2, ESDN
基因ID : (Human) 131566
基因符号 : DCBLD2
蛋白别名 : 1700055P21Rik, coagulation factor V/VIII-homology domains protein 1, CUB, LCCL and coagulation factor V/VIII-homology domains protein 1, Discoidin, CUB and LCCL domain-containing protein 2, Endothelial and smooth muscle cell-derived neuropilin-like protein
UniProt ID (Human) Q96PD2