Mitochondrial myopathy in rats fed with a diet containing beta-guanidine propionic acid, an inhibitor of creatine entry in muscle cells.
In rats with phosphoryl-creatine depletion (fed a standard Randoin-Causeret diet containing 1% beta-guanidine propionic acid) abnormal mitochondria were observed in slow skeletal muscles, often containing paracrystalline inclusions very like those induced by ischaemia or mitochondrial poisons and in human mitochondrial myopathy.
Gori
Z
Z
Istituto di Patologia Generale, II Cattedra, Pisa, Italy.
De Tata
V
V
Pollera
M
M
Bergamini
E
E
D000818:Animals; D001835:Body Weight / Q000187:drug effects; D003401:Creatine / Q000378:metabolism; D006146:Guanidines / Q000633:toxicity*; D008297:Male; D008854:Microscopy, Electron; D008931:Mitochondria, Muscle / Q000187:drug effects* / Q000648:ultrastructure; D009132:Muscles / Q000378:metabolism / Q000648:ultrastructure; D009135:Muscular Diseases / Q000139:chemically induced; D010725:Phosphocreatine / Q000378:metabolism; D011422:Propionates / Q000633:toxicity*; D051381:Rats; D011919:Rats, Inbred Strains; D013997:Time Factors
D006146:Guanidines; D011422:Propionates; D010725:Phosphocreatine; D003401:Creatine; C005775:guanidinopropionic acid