Nerve Conduction Abnormalities and Neuromyotonia in Genetically Engineered Mouse Models of Human Hereditary Neuropathies

Annals of the New York Academy of Sciences
J Zielasek, Klaus V Toyka

Abstract

We performed electrophysiological studies in myelin protein mutant mice in order to characterize nerve conduction changes. We performed neurographic studies on the facial and sciatic nerves and needle electromyography (EMG). Mice homozygously deficient for the peripheral myelin protein 22 gene (Pmp22-/-) exhibited increased motor latencies, reduced nerve conduction velocities, and polyphasia of the M-response, which are the typical electrophysiological signs of dysmyelination. PMP22 +/- mice developed only mild conduction slowing at an old age and a mild reduction of the M-amplitude, which indicates mild axonal dysfunction. Mice overexpressing Pmp22 developed severe electrophysiological signs of dysmyelination. In myelin protein zero-deficient mice (P0-/-), we found alterations similar to those found in Pmp22-/- mice, whereas P0+/- mice developed mildly increased sciatic nerve F-wave latencies only late in life, which indicates only mild dysmyelination. Connexin 32-deficient mice showed electrophysiological evidence of mild axonal damage. By EMG, we found the clinical and electrophysiological signs of neuromyotonia, that is, continuous spontaneous motor unit discharges, often in rhythmic patterns (myokymia), in P0-/-, Pmp22-/-,...Continue Reading

References

Dec 1, 1975·Journal of the Neurological Sciences·P A Low, J G McLeod
Jun 1, 1979·Annals of Neurology·J W LanceJ Pollard
May 15, 1992·Proceedings of the National Academy of Sciences of the United States of America·U SuterE M Shooter
Mar 19, 1992·Nature·U SuterE M Shooter
Sep 1, 1965·Klinische Wochenschrift·H G Mertens, S Zschocke
Jun 1, 1980·Brain : a Journal of Neurology·A E Harding, P K Thomas
Sep 1, 1993·Muscle & Nerve·F E Cardoso, J Jankovic
May 1, 1996·Neuron·M SeredaK A Nave
Sep 3, 1996·Proceedings of the National Academy of Sciences of the United States of America·E NellesK Willecke
Jan 7, 1998·Journal of Neurology, Neurosurgery, and Psychiatry·K V ToykaU Suter
Jan 7, 1998·Journal of Anatomy·R Martini
Feb 11, 1998·Journal of Neurology·M M Reilly
Dec 16, 1998·Current Opinion in Neurology·R MartiniK V Toyka
Nov 1, 1961·Journal of Neurology, Neurosurgery, and Psychiatry·H Isaacs

❮ Previous
Next ❯

Citations

Jun 12, 2014·Journal of Neuropathology and Experimental Neurology·Kevin L SeburnRobert W Burgess
Dec 29, 2020·Neuroscience Letters·Kathryn R MossAhmet Höke

❮ Previous
Next ❯

Related Concepts

Trending Feeds

COVID-19

Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Blastomycosis

Blastomycosis fungal infections spread through inhaling Blastomyces dermatitidis spores. Discover the latest research on blastomycosis fungal infections here.

Nuclear Pore Complex in ALS/FTD

Alterations in nucleocytoplasmic transport, controlled by the nuclear pore complex, may be involved in the pathomechanism underlying multiple neurodegenerative diseases including Amyotrophic Lateral Sclerosis and Frontotemporal Dementia. Here is the latest research on the nuclear pore complex in ALS and FTD.

Applications of Molecular Barcoding

The concept of molecular barcoding is that each original DNA or RNA molecule is attached to a unique sequence barcode. Sequence reads having different barcodes represent different original molecules, while sequence reads having the same barcode are results of PCR duplication from one original molecule. Discover the latest research on molecular barcoding here.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Position Effect Variegation

Position Effect Variagation occurs when a gene is inactivated due to its positioning near heterochromatic regions within a chromosome. Discover the latest research on Position Effect Variagation here.

STING Receptor Agonists

Stimulator of IFN genes (STING) are a group of transmembrane proteins that are involved in the induction of type I interferon that is important in the innate immune response. The stimulation of STING has been an active area of research in the treatment of cancer and infectious diseases. Here is the latest research on STING receptor agonists.

Microbicide

Microbicides are products that can be applied to vaginal or rectal mucosal surfaces with the goal of preventing, or at least significantly reducing, the transmission of sexually transmitted infections. Here is the latest research on microbicides.