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Locomotor and skeletal muscle abnormalities in trembler J neuropathic mice
Author(s) -
Falk Darin J.,
Galatas Tori,
Todd Adrian G.,
Soto Elliott P.,
Harris Andrew B.,
Notterpek Lucia
Publication year - 2018
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.25987
Subject(s) - medicine , skeletal muscle , anatomy , muscle weakness , weakness , peripheral neuropathy , neuropathic pain , pathology , neuroscience , endocrinology , anesthesia , biology , diabetes mellitus
Patients with hereditary peripheral neuropathies exhibit characteristic deformities of the hands and feet and have difficulty ambulating. To examine to what extent neuropathic animals recapitulate these deficits, we studied trembler J (TrJ) mice, which model early‐onset demyelinating neuropathy. Methods A cohort of 4‐month‐old female wild type and neuropathic mice were evaluated for locomotor measurements, neuromuscular function, and skeletal muscle proteolysis and morphometry. Results Utilizing the DigiGait imaging system, we identified pronounced alterations in forepaw and hindpaw angles and a decrease in hindpaw area on the treadmill in neuropathic rodents. Torque production by the tibialis anterior (TA) muscle was significantly weakened and was paralleled by a decrease in myofiber cross‐sectional area and an increase in muscle tissue proteolysis. Discussion Our findings in TrJ mice reflect the phenotypic presentation of the human neuropathy in which patients exhibit weakness of the TA muscle resulting in foot drop and locomotor abnormalities. Muscle Nerve 57 : 664–671, 2018