Hereditary motor end-plate disease in the mouse: light and electron microscopic studies.

Duchen, L. W.

doi:10.1136/jnnp.33.2.238

Cited by 78 publications

(46 citation statements)

References 15 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…In the original motor endplate disease (med) mouse (Scn8a med ) described previously by Duchen (1970), a null mutation in Scn8a results in reduced neuromuscular transmission leading to paralysis and lethality approximately at the time of weaning (Burgess et al, 1995). Nerve conduction velocity is reduced and the refractory period is prolonged in these animals (Angaut-Petit et al, 1982).…”

Section: Introductionmentioning

confidence: 99%

Physiological Maturation of Photoreceptors Depends on the Voltage-Gated Sodium Channel Na_V1.6 (Scn8a)

Côté¹,

Repentigny²,

Coupland³

et al. 2005

J. Neurosci.

View full text Add to dashboard Cite

Voltage-gated sodium channels (VGSCs) ensure the saltatory propagation of action potentials along axons by acting as signal amplifiers at the nodes of Ranvier. In the retina, activity mediated by VGSCs is important for the refinement of the retinotectal map. Here, we conducted a full-field electroretinogram (ERG) study on mice null for the sodium channel Na V 1.6. Interestingly, the light-activated hyperpolarization of photoreceptor cells (the a-wave) and the major "downstream" components of the ERG, the b-wave and the oscillatory potentials, are markedly reduced and delayed in these mice. The functional deficit was not associated with any morphological abnormality. We demonstrate that Scn8a is expressed in the ganglion and inner nuclear layers and at low levels in the outer nuclear layer beginning shortly before the observed ERG deficit. Together, our data reveal a previously unappreciated role for VGSCs in the physiological maturation of photoreceptors.

show abstract

Section: Introductionmentioning

confidence: 99%

Physiological Maturation of Photoreceptors Depends on the Voltage-Gated Sodium Channel Na_V1.6 (Scn8a)

Côté¹,

Repentigny²,

Coupland³

et al. 2005

J. Neurosci.

View full text Add to dashboard Cite

show abstract

“…These small blocks are fused together into a large block by pouring on molten wax (Beesley & Daniel, 1956). This serial block method has been much used with mice for examining the whole spinal column, with cord in situ (Duchen, 1970).…”

Section: Pmentioning

confidence: 99%

Demonstrations

1979

The Journal of Physiology

View full text Add to dashboard Cite

DEMONSTRATIONSThe collection of saliva in the new-born rabbit, guinea-pig and rat BY SYLViA L. PULL AND J. M. REYNOLDS. Royal Dental School, St George'8 Hospital Medical School, Cranmer Terrace, London SW17 ORE The characteristics of the developing salivary gland have been chiefly suggested from morphological studies. As an alternative approach, some methods for the physiological study of the developing gland were demonstrated.Neonatal rabbits were anaesthetized with 25 % urethane in saline, 175 g kg-' i.P.or via an ear vein. A femoral vein and, where possible, a femoral artery were cannulated using nylon tubing (Portex PP202) drawn down to outside diameters of 60-150 ,um. Anaesthesia was maintained intravenously and the arterial cannula used

show abstract

“…Affected mice usually die at about 20 days of age. The results of electrophysiological studies (Duchen & Stefani, 1971;Harris & Ward, 1974) which utilized intracellular recording techniques on biceps brachii nerve-muscle preparations, preparations which had previously been shown in a histological survey to be the most severely affected in motor end-plate diseased mice (Duchen, 1970), suggested that this malady is caused by a failure of peripheral motor nerve action potentials to invade motor nerve terminals. Low (depolarized) resting potentials, tetrodotoxin resistant action potentials, spontaneous fibrillations, and extrajunctional supersensitivity to acetylcholine were found in diseased muscle.…”

Section: Introductionmentioning

confidence: 99%

“…This disease is a hereditary (autosomal recessive) neuromuscular disorder which is characterized by a progressive muscular weakness appearing from 8 to 10 days after birth (Duchen, 1970). Affected mice usually die at about 20 days of age.…”

Section: Introductionmentioning

confidence: 99%

A comparative electrophysiological study of motor end‐plate diseased skeletal muscle in the mouse.

Weinstein¹

1980

The Journal of Physiology

View full text Add to dashboard Cite

SUMMARY1. Experiments using intracellular recording, stimulation, and microionophoretic techniques were performed on extensor digitorum longus nerve-muscle preparations excised from mice having hereditary 'motor end-plate disease'. Control experiments were performed on normal innervated and chronically denervated nerve-muscle preparations.2. Two physiologically distinct groups of muscle fibres were found in the diseased muscles. Group I is similar to normal innervated muscle with respect to resting potentials, cable properties, neuromuscular transmission, miniature end-plate potentials, and extrajunctional acetylcholine sensitivity. Group II is similar to denervated muscle in the above respects except that (i) neuromuscular transmission, though abnormal, was present, and (ii) miniature end-plate potentials (m.e.p.p.s), often having large amplitudes, were found in these muscle fibres.3. Large m.e.p.p.s appear to be due to an increase in muscle fibre input resistance and to the quantal release of abnormally large amounts of acetylcholine from motor nerve terminals.4. Nerve stimulation of Group II muscle fibres evoked action potentials with a delayed repolarization phase, suggesting that a prolonged acetylcholine-induced conductance change occurs at motor end-plates.5. Neuromuscular physiology in motor end-plate disease is similar to that reported for frog nerve-muscle preparations which have been incubated in high Ca2+ Ringer.

show abstract

Hereditary motor end-plate disease in the mouse: light and electron microscopic studies.

Cited by 78 publications

References 15 publications

Physiological Maturation of Photoreceptors Depends on the Voltage-Gated Sodium Channel Na_V1.6 (Scn8a)

Physiological Maturation of Photoreceptors Depends on the Voltage-Gated Sodium Channel Na_V1.6 (Scn8a)

Demonstrations

A comparative electrophysiological study of motor end‐plate diseased skeletal muscle in the mouse.

Contact Info

Product

Resources

About

Hereditary motor end-plate disease in the mouse: light and electron microscopic studies.

Cited by 78 publications

References 15 publications

Physiological Maturation of Photoreceptors Depends on the Voltage-Gated Sodium Channel NaV1.6 (Scn8a)

Physiological Maturation of Photoreceptors Depends on the Voltage-Gated Sodium Channel NaV1.6 (Scn8a)

Demonstrations

A comparative electrophysiological study of motor end‐plate diseased skeletal muscle in the mouse.

Contact Info

Product

Resources

About

Physiological Maturation of Photoreceptors Depends on the Voltage-Gated Sodium Channel Na_V1.6 (Scn8a)

Physiological Maturation of Photoreceptors Depends on the Voltage-Gated Sodium Channel Na_V1.6 (Scn8a)