A fatal, systemic mitochondrial disease with decreased mitochondrial enzyme activities, abnormal ultrastructure of the mitochondria and deficiency of heat shock protein-60. - Agsteribbe,E.; Huckriede,A.; Veenhuis,M.; Ruiters,M.HJ.; Niezen-Koning,K.E.; Skjeldal,O.H. (Biochem Biophys Res Commun, 1993)