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000602498 1001_ $$aSonani, Ravi R.$$b0
000602498 245__ $$aCrystal structures of apo- and FAD-bound human peroxisomal acyl-CoA oxidase provide mechanistic basis explaining clinical observations
000602498 260__ $$aNew York, NY [u.a.]$$bElsevier$$c2022
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000602498 520__ $$aPeroxisomal acyl-CoA oxidase 1a (ACOX1a) catalyzes the first and rate-limiting step of fatty acid oxidation, the conversion of acyl-CoAs to 2-trans-enoyl-CoAs. The dysfunction of human ACOX1a (hACOX1a) leads to deterioration of the nervous system manifesting in myeloneuropathy, hypotonia and convulsions. Crystal structures of hACOX1a in apo- and cofactor (FAD)-bound forms were solved at 2.00 and 2.09 Å resolution, respectively. hACOX1a exists as a homo-dimer with solvation free energy gain (ΔGo) of −44.7 kcal mol−1. Two FAD molecules bind at the interface of protein monomers completing the active sites. The substrate binding cleft of hACOX1a is wider compared to human mitochondrial very-long chain specific acyl-CoA dehydrogenase. Mutations (p.G178C, p.M278V and p.N237S) reported to cause dysfunctionality of hACOX1a are analyzed on its 3D-structure to understand structure-function related perturbations and explain the associated phenotypes.
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000602498 7001_ $$aBlat, Artur$$b1
000602498 7001_ $$0P:(DE-H253)PIP1021406$$aDubin, Grzegorz$$b2$$eCorresponding author
000602498 773__ $$0PERI:(DE-600)1483284-7$$a10.1016/j.ijbiomac.2022.02.008$$gVol. 205, p. 203 - 210$$p203 - 210$$tInternational journal of biological macromolecules$$v205$$x0141-8130$$y2022
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