Axonal degeneration in the Trembler-j mouse demonstrated by stimulated single-fiber electromyography

Muscle and Nerve

Volumn 36, Issue 1, 2007, Pages 81-86

  Meekins, Gregg D ^a   Carter, Gregory T ^b   Emery, Michael J ^b   Weiss, Michael D ^a  

^a UNIVERSITY OF WASHINGTON MEDICAL CENTER   (United States)

^b UNIVERSITY OF WASHINGTON SCHOOL OF MEDICINE   (United States)

Author keywords

Axonal degeneration; Demyelinating neuropathy; Jitter; Single fiber EMG; Trembler j

Indexed keywords

PERIPHERAL MYELIN PROTEIN 22;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; CONTROLLED STUDY; DEMYELINATION; ELECTROMYOGRAM; HEREDITARY MOTOR SENSORY NEUROPATHY; MOUSE; MOUSE MUTANT; MOUSE STRAIN; MUSCLE REINNERVATION; NERVE FIBER DEGENERATION; NEUROMUSCULAR TRANSMISSION; NONHUMAN; POINT MUTATION; PRIORITY JOURNAL; WILD TYPE;

ACTION POTENTIALS; AGE FACTORS; ANIMALS; CHOLINESTERASE INHIBITORS; DISEASE MODELS, ANIMAL; DNA MUTATIONAL ANALYSIS; ELECTRIC STIMULATION; ELECTROMYOGRAPHY; MICE; MICE, INBRED C57BL; MICE, NEUROLOGIC MUTANTS; MUSCLE FIBERS; MUTATION; MYELIN PROTEINS; NEOSTIGMINE; NERVE DEGENERATION;

EID: 34347328180     PISSN: 0148639X     EISSN: 10974598     Source Type: Journal    
DOI: 10.1002/mus.20786     Document Type: Article

References (21)

1. Berman PW, Patrick J. Experimental myasthenia gravis. A murine system. J Exp Med 1980;151:204-223.
2. Bertorini TE, Stalberg E, Yuson CP, Engel WK. Single-fiber electromyography in neuromuscular disorders: correlation of muscle histochemistry, single-fiber electromyography, and clinical findings. Muscle Nerve 1994;17:345-353.
3. Gooch CL, Mosier DR. Stimulated single fiber electromyography in the mouse: techniques and normative data. Muscle Nerve 2001;24:941-945.
4. Henry EW, Cowen JS, Sidman RL. Comparison of the Trembler and Trembler-J mouse phenotypes: varying severity of peripheral hypomyelination. J Neuropathol Exp Neurol 1983; 42:688-706.
5. Meekins GD, Emery MJ, Weiss MD. Nerve conduction abnormalities in the trembler-j mouse: a model for Charcot-Marie-Tooth disease type 1A? J Periph Nerv Syst 2004;9:177-182.
6. Meekins GD, Weiss MD. Electrodiagnostic studies in a murine model of demyelinating Charcot-Marie-Tooth disease. Phys Med Rehabil Clin North Am 2005;16:967-979.
7. Oh SJ. The single-fiber EMG in chronic demyelinating neuropathy. Muscle Nerve 1989;12:371-377.
8. Robertson AM, King RHM, Muddle JR, Thomas PK. Abnormal Schwann cell/axon interactions in the Trembler-j mouse. J Anal 1997;190:423-432.
9. Robertson AM, Huxley C, King RHM, Thomas PK. Development of early postnatal peripheral nerve abnormalities in Trembler-J and PMP-22 transgenic mice. J Anat 1999;195: 331-339.
10. Russell JW, Windebank AJ, Harper CM Jr. Treatment of stable chronic demyelinating polyneuropathy with 3,4-diaminopyridine. Mayo Clin Proc 1995;70:532-539.
11. Schiller HH, Stalberg E, Schwarz MS. Regional curare for the reduction of the safety factor in human motor end-plates studied with single liber electromyography. J Neurol Neurosurg Psychiatry 1975;38:805-809.
12. Sidman RL, Cowen JS, Eicher EM. Inherited muscle and nerve diseases in mice: tabulation with commentary. Ann N Y Acad Sci 1979;317:494-505.
13. Stalberg E, Ekstedt J, Broman A. The electromyographic jitter in normal human muscles. Electroencephalogr Clin Neurophysiol 1971;31:429-438.
14. Stalberg E, Schiller HH, Schwartz MS. Safely factor in single human motor end plates studied in vivo with single fiber electromyography. J Neurol Nevirosurg Psychiatry 1975;38: 799-804.
15. Stalberg E, Schwartz MS, Trontelj JV. Single fiber electromyography in various processes affecting the anterior horn cell. J Neurol Sci 1975;24:403-415.
16. Suter U, Moskow JJ, Welcher AA, Snipes GJ, Kosaras B, Sidman RL, et al. A leucine-to-proline mutation in the putative first transmembrane domain of the 22-kDa peripheral myelin protein in the trembler-J mouse. Proc Natl Acad Sci U S A 1992;89:4382-4386.
17. Thiele B, Stalberg E. Single fiber EMG findings iu polyneuropathies of different aetiology. J Neurol Neurosurg Psychiatry 1975;38:881-887.
18. Trontelj JV, Mihelin M, Fernandez JM, Stalberg E. Axonal stimulation for end plate jitter studies. J Neurol Neurosurg Psychiatry 1986;49:677-685.
19. Trontelj JV, Stalberg E, Mihelin M, Khuraibet A. Jitter of the simulated motor axon. Muscle Nerve 1992;15:449-454.
20. Valentijn LJ, Baas F, Wolterman RA, Hoogendijk JE, van den Bosch NH, Zom I, et al. Identical point mutations of PMP-22 in Trembler-J mouse and Charcot-Marie-Tooth disease type 1A. Nat Genet 1992;2:288-291.
21. Zielasek J, Toyka KV. Nerve conduction abnormalities and neuromyotonia in genetically engineered mouse models of human hereditary neuropathies. Ann NY Acad Sci 1999;883: 310-320.