Effects of low doses of glycosaminoglycans and insulin-like growth factor-I on motor neuron disease in wobbler mouse.
In this study we examined the effects of insulin-like growth factor-I (IGF-I) and of glycosaminoglycans (GAGs) on the progressive motor neuron disease in wobbler mice. After clinical diagnosis at age 3 weeks, mice received daily subcutaneous injections of IGF-I, or GAGs, or saline for 3 weeks. The histometric analysis revealed that biceps muscle fiber diameter was reduced in wobbler mice and that treatments with GAGs and IGF-I prevented such a drop. The number of atrophic small fibers was markedly reduced and that of the larger ones augmented. No effects on body growth and biceps muscle weight were observed. The combined AChE-silver staining revealed that both treatments promoted intramuscular axonal sprouting. The typical decline of grip strength in wobbler mice was also prevented. This study suggests that GAGs and IGF-I administrations can retard the onset of motor deficit, and reduce muscle atrophy in wobbler mice.
Vergani
L
L
Department of Pharmacology, Chemotherapy and Medical Toxicology, University of Milano, Italy.
Finco
C
C
Di Giulio
A M
AM
Muller
E E
EE
Gorio
A
A
D000110:Acetylcholinesterase / Q000378:metabolism; D000818:Animals; D006025:Glycosaminoglycans / Q000008:administration & dosage* / Q000494:pharmacology; D018737:Hand Strength; D007334:Insulin-Like Growth Factor I / Q000008:administration & dosage* / Q000494:pharmacology; D051379:Mice; D008818:Mice, Neurologic Mutants; D016472:Motor Neuron Disease / Q000235:genetics / Q000473:pathology* / Q000503:physiopathology*; D018485:Muscle Fibers, Skeletal / Q000187:drug effects / Q000648:ultrastructure; D009132:Muscles / Q000187:drug effects / Q000201:enzymology / Q000473:pathology; D013194:Staining and Labeling
D006025:Glycosaminoglycans; D007334:Insulin-Like Growth Factor I; D000110:Acetylcholinesterase