Karnitin O-palmitoiltransferaza 2, mitohondrijska je enzim koji je kod čoveka kodiran CPT2genom.[1][2]
Prekurzor karnitin palmitoiltransferaze II (CPT2) je nuklearni protein koji se transportuje do mitohondrijske unutrašnje membrane inner membrane. CPT2 zajedno sa karnitin palmitoiltransferazom I oksidizuje dugoloančane masne kiseline u mitohondrijama. Defekti ovog gena uzrokuje poremećaje u mitohondrijskoj oksidaciji dugolančanih masnih kiselina (LCFA) i deficijenciju karnitin palmitoiltrasferaze II.[2]
Acil-KoA iz citozola do mitohodrijkog matriksa
Reference
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