Effect of denervation on the resting membrane potential of healthy and dystrophic muscle.

Lenman, J. A. R.

doi:10.1136/jnnp.28.6.525

Cited by 22 publications

(5 citation statements)

References 12 publications

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“…Androgens apparently exert their inhibitory influence on spontaneous release by regulating a mechanism yet to be identified that links presynaptic calcium channels to the transmitter release machinery (33). Protecting this mechanism from the deleterious effects of androgens in SBMA may offer a way to protect quantal release, which in turn may prevent muscle fibers from compensating to increase their excitability by reducing their RMP (35,36). An important next step is to identify this potentially pivotal early mechanism that may trigger further pathogenesis.…”

Section: Amplification Of Normal Ar Action May Underlie Some Aspects Of Diseasementioning

confidence: 99%

Pre-clinical symptoms of SBMA may not be androgen-dependent: implications from two SBMA mouse models

Halievski

Katsuno

et al. 2018

Human Molecular Genetics

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A distinguishing aspect of spinal and bulbar muscular atrophy (SBMA) is its androgen-dependence, possibly explaining why only males are clinically affected. This disease, which impairs neuromuscular function, is linked to a polyglutamine expansion mutation in the androgen receptor (AR). In mouse models of SBMA, motor dysfunction is associated with pronounced defects in neuromuscular transmission, including defects in evoked transmitter release (quantal content, QC) and fiber membrane excitability (based on the resting membrane potential, RMP). However, whether such defects are androgen-dependent is unknown. Thus, we recorded synaptic potentials intracellularly from adult muscle fibers of transgenic (Tg) AR97Q male mice castrated pre-symptomatically. Although castration largely protects both QC and the RMP of fibers, correlating with the protective effect of castration on motor function, significant deficits in QC and RMP remained. Surprisingly, comparable defects in QC and RMP were also observed in pre-symptomatic AR97Q males, indicating that such defects emerge early and are pre-clinical. Exposing asymptomatic Tg females to androgens also induces both motor dysfunction and comparable defects in QC and RMP. Notably, asymptomatic Tg females also showed significant deficits in QC and RMP, albeit less severe, supporting their pre-clinical nature, but also raising questions about the androgen-dependence of pre-clinical symptoms. In summary, current evidence indicates that disease progression depends on androgens, but early pathogenic events may be triggered by the mutant AR allele independent of androgens. Such early, androgen-independent disease mechanisms may also be relevant to females carrying the SBMA allele.

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Section: Amplification Of Normal Ar Action May Underlie Some Aspects Of Diseasementioning

confidence: 99%

Pre-clinical symptoms of SBMA may not be androgen-dependent: implications from two SBMA mouse models

Halievski

Katsuno

et al. 2018

Human Molecular Genetics

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“…It remains to be proved whether the basic disturbance is the persistent sequestration of the cation in the sarcoplasmic reticulum, as speculated by Howell, Fairhurst, and Jenden (1966), or the facilitation of the calcium carried to and from this (aI) (bk H. Radu, I. Godri, E. Albu, A. Radu, and R. Robu structure. Nevertheless, the hypothetical hypersensitivity of this sarcoplasmic reticulum packed with calcium may be correlated with the electrophysiological data of Lenman (1965) and McComas and Mrozek (1967) concerning the significant reduction of the resting potential in denervated muscle, and also to the findings of Nastuk and Liu (1966) relating to changes in chemosensitivity of muscle postjunctional m-mbrane produced by calcium. In addition Wechsler (1966) showed that the sarcoplasmic reticulum in human denervated muscle remains relatively preserved morphologically.…”

Section: Discussionmentioning

confidence: 60%

Calcium uptake and bioelectrical activity of denervated and myotonic muscle

Radu¹,

Gödri²,

Albu³

et al. 1970

Journal of Neurology, Neurosurgery & Psychiatry

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“…Only in the gastrocnemius muscle did the distribution of membrane potentials show a slightly wider range of values in the dystrophic than in the normal hamster which compares with the very wide scatter ofl values seen in the dystrophic mouse (Lenman, 1965). In a study of excised muscle in the hamster Harris and Ward (1975) *o ib a small but significant decrease in resting membrane potential in the dystrophic compared with normal animals.…”

Section: Discussionmentioning

confidence: 88%

Muscle fibre excitability and resting membrane potential in hamster muscular dystrophy.

Lenman¹,

Tulley²

1979

Journal of Neurology, Neurosurgery & Psychiatry

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Effect of denervation on the resting membrane potential of healthy and dystrophic muscle.

Cited by 22 publications

References 12 publications

Pre-clinical symptoms of SBMA may not be androgen-dependent: implications from two SBMA mouse models

Pre-clinical symptoms of SBMA may not be androgen-dependent: implications from two SBMA mouse models

Calcium uptake and bioelectrical activity of denervated and myotonic muscle

Muscle fibre excitability and resting membrane potential in hamster muscular dystrophy.

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