[HTML][HTML] Rapid degradation of short-chain acyl-CoA dehydrogenase variants with temperature-sensitive folding defects occurs after import into mitochondria
TJ Corydon, P Bross, TG Jensen, MJ Corydon… - Journal of Biological …, 1998 - ASBMB
Most disease-causing missense mutations in short-chain acyl-CoA dehydrogenase (SCAD)
and medium-chain acyl-CoA dehydrogenase are thought to compromise the mitochondrial
folding and/or stability of the mutant proteins. To address this question, we studied the
biogenesis of SCAD proteins in COS-7 cells transfected with cDNA corresponding to two
SCAD missense mutations, R22W (identified in a patient with SCAD deficiency) or R22C
(homologous to a disease-associated R28C mutation in medium-chain acyl-CoA …
and medium-chain acyl-CoA dehydrogenase are thought to compromise the mitochondrial
folding and/or stability of the mutant proteins. To address this question, we studied the
biogenesis of SCAD proteins in COS-7 cells transfected with cDNA corresponding to two
SCAD missense mutations, R22W (identified in a patient with SCAD deficiency) or R22C
(homologous to a disease-associated R28C mutation in medium-chain acyl-CoA …