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Publication : Down's syndrome: morphological remodelling and increased complexity in the neuromuscular junction of transgenic CuZn-superoxide dismutase mice.

First Author  Avraham KB Year  1991
Journal  J Neurocytol Volume  20
Issue  3 Pages  208-15
PubMed ID  1828079 Mgi Jnum  J:81469
Mgi Id  MGI:2449387 Doi  10.1007/BF01186993
Citation  Avraham KB, et al. (1991) Down's syndrome: morphological remodelling and increased complexity in the neuromuscular junction of transgenic CuZn-superoxide dismutase mice. J Neurocytol 20(3):208-15
abstractText  Transgenic mice carrying the human CuZn-superoxide dismutase gene were used to investigate whether CuZn-superoxide dismutase gene dosage is involved in the signs of neuromuscular junction deterioration associated with Down's syndrome. Three parameters of neuromuscular junction morphology were studied in hindlimb muscles of CuZn-superoxide dismutase-transgenic mice and their non-transgenic littermates: nerve terminal length, number of nerve terminal branching points and incidence of sprouting that results in synapse formation. These parameters increased with advanced age and the increase occurred earlier in CuZn-superoxide dismutase-transgenic mice. Therefore, the data is in line with the possibility that CuZn-superoxide dismutase-transgenic mice are undergoing premature ageing with respect to neuromuscular junction morphology, most probably owing to a gene dosage effect of CuZn-superoxide dismutase.
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