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Thermal unfolding of medium-chain acyl-CoA dehydrogenase and iso(3)valeryl-CoA dehydrogenase : study of the effect of genetic defects on enzyme stability

Thermal unfolding of medium-chain acyl-CoA dehydrogenase and iso(3)valeryl-CoA dehydrogenase : study of the effect of genetic defects on enzyme stability

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NASSER, Ibrahim, Al-Walid MOHSEN, Ilian JELESAROV, Jerry VOCKLEY, Peter MACHEROUX, Sandro GHISLA, 2004. Thermal unfolding of medium-chain acyl-CoA dehydrogenase and iso(3)valeryl-CoA dehydrogenase : study of the effect of genetic defects on enzyme stability. In: Biochimica et Biophysica Acta. 1690(1), pp. 22-32. ISSN 0925-4439

@article{Nasser2004Therm-8321, title={Thermal unfolding of medium-chain acyl-CoA dehydrogenase and iso(3)valeryl-CoA dehydrogenase : study of the effect of genetic defects on enzyme stability}, year={2004}, number={1}, volume={1690}, issn={0925-4439}, journal={Biochimica et Biophysica Acta}, pages={22--32}, author={Nasser, Ibrahim and Mohsen, Al-Walid and Jelesarov, Ilian and Vockley, Jerry and Macheroux, Peter and Ghisla, Sandro} }

<rdf:RDF xmlns:rdf="http://www.w3.org/1999/02/22-rdf-syntax-ns#" xmlns:bibo="http://purl.org/ontology/bibo/" xmlns:dc="http://purl.org/dc/elements/1.1/" xmlns:dcterms="http://purl.org/dc/terms/" xmlns:xsd="http://www.w3.org/2001/XMLSchema#" > <rdf:Description rdf:about="https://kops.uni-konstanz.de/rdf/resource/123456789/8321"> <dc:creator>Jelesarov, Ilian</dc:creator> <dc:date rdf:datatype="http://www.w3.org/2001/XMLSchema#dateTime">2011-03-24T17:42:39Z</dc:date> <bibo:uri rdf:resource="http://kops.uni-konstanz.de/handle/123456789/8321"/> <dc:creator>Mohsen, Al-Walid</dc:creator> <dcterms:title>Thermal unfolding of medium-chain acyl-CoA dehydrogenase and iso(3)valeryl-CoA dehydrogenase : study of the effect of genetic defects on enzyme stability</dcterms:title> <dc:contributor>Macheroux, Peter</dc:contributor> <dc:creator>Vockley, Jerry</dc:creator> <dc:format>application/pdf</dc:format> <dcterms:available rdf:datatype="http://www.w3.org/2001/XMLSchema#dateTime">2011-03-24T17:42:39Z</dcterms:available> <dc:creator>Macheroux, Peter</dc:creator> <dc:contributor>Jelesarov, Ilian</dc:contributor> <dcterms:rights rdf:resource="https://creativecommons.org/licenses/by-nc-nd/2.0/legalcode"/> <dcterms:issued>2004</dcterms:issued> <dcterms:abstract xml:lang="eng">Genetic defects affecting acyl-CoA dehydrogenases (ACAD) key enzymes in the degradation of fatty acids and branched chain amino acids are increasingly recognized as being more widespread than originally thought. For the medium-chain acyl-CoA dehydrogenase (MCAD), the K304E mutation is the most common genetic defect among Caucasian populations. The effect of substrate or substrate analog binding on the stability of wild-type MCAD and isovaleryl-CoA dehydrogenase (i3VD) and their genetic mutants (K304E- and T168A-MCAD and A282V-i3VD) is examined. Binding to the mutant ACADs is generally ≈10-fold weaker compared to wild-type proteins. Thermal stability of wt-MCAD (melting point ≈53.6 °C) is significantly higher compared to wt-i3VD (≈49.3 °C). With the exception of the A282V-i3VD mutant, a high degree of stabilization (5 11 °C) is induced by conversion into the reduced enzyme form complexed with product. The results are discussed based on the 3D-structures of the enzymes, and it is concluded that in the case of K304E-MCAD thermal stability as such is not a major contribution to the clinical phenotype. With the T168A-MCAD and A282V-i3VD mutants, however, the diminished thermal stability and minor stabilization by ligands must be regarded as an important factor contributing to the manifestation of the disease.</dcterms:abstract> <dc:contributor>Ghisla, Sandro</dc:contributor> <dc:rights>deposit-license</dc:rights> <dc:creator>Ghisla, Sandro</dc:creator> <dc:contributor>Vockley, Jerry</dc:contributor> <dc:contributor>Nasser, Ibrahim</dc:contributor> <dc:contributor>Mohsen, Al-Walid</dc:contributor> <dcterms:bibliographicCitation>First publ. in: Biochimica et Biophysica Acta / Molecular basis of disease, 1690 (2004), 1, pp. 22-32</dcterms:bibliographicCitation> <dc:language>eng</dc:language> <dc:creator>Nasser, Ibrahim</dc:creator> </rdf:Description> </rdf:RDF>

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