Progressive Ophthalmoplegia, Glycogen Storage, and Abnormal Mitochondria

An Ocular Myopathy with Glycogen Storage and Abnormal Mitochondria in Muscle Fibres

“…In the case of progressive oph thalmoplegia with glycogen storage described by Di Mauro et al [1], which presents many findings similar to our case, the studies perfomed on isolated mitochondria show reduc tion of respiratory rate, with possible slow down of glycolysis and glycogen accumula tion in muscle fibres. This hypothesis is supported by others [5], explaining the muscle glycogen accumulation in a familial mitochondrial myopathy associ ated with disordered oxidative metabolism.…”

“…Nev ertheless some cases have been characterized by the singular associated appearance of mi tochondrial changes with glycogen accumula tion in muscle fibres [1,5,7,9].…”

Skeletal Muscle Calcium Metabolism and Contractile Force in Vitamin D-deficient Chicks

“…The suispentsionl was cenitrifuigedI at 6,600 rpm (JA 20 rotor) for 10 mimi att 50C, aind( the mitochond(Irial pellet was r-estispend(le( in 0.25 \I stucrose and 2 mMN1 Hepes-Tris (pH 7.4). Portioins of the suspenisioin were taken for electron imicroscopy ats previously dlescribed (10), proteini assay (15), anl for polarographic imieatsuiremilenits of oxygen uptake (19).…”

Polyneuropathy in Progressive External Ophthalmoplegia

“…A deficit of the energy metabolism that is assumed to underlie the overall manifesta tions of PEO [7,[20][21][22] can indeed cause damage of the peripheral nerves [23], even though this seems to occur infrequently, at least in a clinically obvious form.…”