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Apolipoprotein CII (ApoCII) is a 79-amino acid protein encoded by the APOC2 gene, serving as an essential cofactor for lipoprotein lipase (LPL) activation. This protein circulates in plasma as a component of triglyceride-rich lipoproteins (TRLs), including very low-density lipoproteins (VLDL) and chylomicrons, with normal concentrations ranging from 3–8 mg per 100 mL. ApoCII binds to LPL via its C-terminal α-helix (residues 58–79), stabilizing the enzyme’s catalytic domain and facilitating the hydrolysis of VLDL triglycerides into free fatty acids and glycerol, a process critical for generating intermediate-density lipoproteins (IDL). This lipolytic activity enables cellular energy uptake and regulates plasma lipid homeostasis.
Deficiency in ApoCII, caused by biallelic APOC2 mutations, results in familial hyperlipoproteinemia type IB, characterized by severe hypertriglyceridemia (>1,000 mg/dL), recurrent pancreatitis, and eruptive xanthomas. The absence of functional ApoCII impairs LPL-mediated triglyceride clearance, leading to chylomicronemia and atherosclerosis in preclinical models. Heterozygotes typically remain asymptomatic due to ApoCII’s excess physiological presence. Clinically, ApoCII deficiency is diagnosed through genetic testing and plasma apolipoprotein profiling, with acute pancreatitis managed via fresh frozen plasma infusions to replenish ApoCII.
Common uses include lipid metabolism studies, cardiovascular research.
Not a hazardous substance or mixture.
11 - Combustible Solids
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