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The wobbler mouse, an ALS animal model

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The wobbler mouse, an ALS animal model. / Moser, Jakob Maximilian; Bigini, Paolo; Schmitt-John, Thomas.

In: Molecular Genetics and Genomics, Vol. 288, No. 5-6, 06.2013, p. 207–229.

Research output: Contribution to journal/Conference contribution in journal/Contribution to newspaperReviewResearchpeer-review

Harvard

Moser, JM, Bigini, P & Schmitt-John, T 2013, 'The wobbler mouse, an ALS animal model', Molecular Genetics and Genomics, vol. 288, no. 5-6, pp. 207–229. https://doi.org/10.1007/s00438-013-0741-0

APA

Moser, J. M., Bigini, P., & Schmitt-John, T. (2013). The wobbler mouse, an ALS animal model. Molecular Genetics and Genomics, 288(5-6), 207–229. https://doi.org/10.1007/s00438-013-0741-0

CBE

Moser JM, Bigini P, Schmitt-John T. 2013. The wobbler mouse, an ALS animal model. Molecular Genetics and Genomics. 288(5-6):207–229. https://doi.org/10.1007/s00438-013-0741-0

MLA

Moser, Jakob Maximilian, Paolo Bigini and Thomas Schmitt-John. "The wobbler mouse, an ALS animal model". Molecular Genetics and Genomics. 2013, 288(5-6). 207–229. https://doi.org/10.1007/s00438-013-0741-0

Vancouver

Moser JM, Bigini P, Schmitt-John T. The wobbler mouse, an ALS animal model. Molecular Genetics and Genomics. 2013 Jun;288(5-6):207–229. https://doi.org/10.1007/s00438-013-0741-0

Author

Moser, Jakob Maximilian ; Bigini, Paolo ; Schmitt-John, Thomas. / The wobbler mouse, an ALS animal model. In: Molecular Genetics and Genomics. 2013 ; Vol. 288, No. 5-6. pp. 207–229.

Bibtex

@article{56a321fdc7914ad192b57223ee38987a,
title = "The wobbler mouse, an ALS animal model",
abstract = "This review article is focused on the research progress made utilizing the wobbler mouse as animal model for human motor neuron diseases, especially the amyotrophic lateral sclerosis (ALS). The wobbler mouse develops progressive degeneration of upper and lower motor neurons and shows striking similarities to ALS. The cellular effects of the wobbler mutation, cellular transport defects, neurofilament aggregation, neuronal hyperexcitability and neuroinflammation closely resemble human ALS. Now, 57 years after the first report on the wobbler mouse we summarize the progress made in understanding the disease mechanism and testing various therapeutic approaches and discuss the relevance of these advances for human ALS. The identification of the causative mutation linking the wobbler mutation to a vesicle transport factor and the research focussed on the cellular basis and the therapeutic treatment of the wobbler motor neuron degeneration has shed new light on the molecular pathology of the disease and might contribute to the understanding the complexity of ALS",
keywords = "ALS, Wobbler, Motor neuron degeneration, GARP complex, Vesicle tethering",
author = "Moser, {Jakob Maximilian} and Paolo Bigini and Thomas Schmitt-John",
year = "2013",
month = jun,
doi = "10.1007/s00438-013-0741-0",
language = "English",
volume = "288",
pages = "207–229",
journal = "Molecular Genetics and Genomics",
issn = "1617-4615",
publisher = "Springer",
number = "5-6",

}

RIS

TY - JOUR

T1 - The wobbler mouse, an ALS animal model

AU - Moser, Jakob Maximilian

AU - Bigini, Paolo

AU - Schmitt-John, Thomas

PY - 2013/6

Y1 - 2013/6

N2 - This review article is focused on the research progress made utilizing the wobbler mouse as animal model for human motor neuron diseases, especially the amyotrophic lateral sclerosis (ALS). The wobbler mouse develops progressive degeneration of upper and lower motor neurons and shows striking similarities to ALS. The cellular effects of the wobbler mutation, cellular transport defects, neurofilament aggregation, neuronal hyperexcitability and neuroinflammation closely resemble human ALS. Now, 57 years after the first report on the wobbler mouse we summarize the progress made in understanding the disease mechanism and testing various therapeutic approaches and discuss the relevance of these advances for human ALS. The identification of the causative mutation linking the wobbler mutation to a vesicle transport factor and the research focussed on the cellular basis and the therapeutic treatment of the wobbler motor neuron degeneration has shed new light on the molecular pathology of the disease and might contribute to the understanding the complexity of ALS

AB - This review article is focused on the research progress made utilizing the wobbler mouse as animal model for human motor neuron diseases, especially the amyotrophic lateral sclerosis (ALS). The wobbler mouse develops progressive degeneration of upper and lower motor neurons and shows striking similarities to ALS. The cellular effects of the wobbler mutation, cellular transport defects, neurofilament aggregation, neuronal hyperexcitability and neuroinflammation closely resemble human ALS. Now, 57 years after the first report on the wobbler mouse we summarize the progress made in understanding the disease mechanism and testing various therapeutic approaches and discuss the relevance of these advances for human ALS. The identification of the causative mutation linking the wobbler mutation to a vesicle transport factor and the research focussed on the cellular basis and the therapeutic treatment of the wobbler motor neuron degeneration has shed new light on the molecular pathology of the disease and might contribute to the understanding the complexity of ALS

KW - ALS

KW - Wobbler

KW - Motor neuron degeneration

KW - GARP complex

KW - Vesicle tethering

U2 - 10.1007/s00438-013-0741-0

DO - 10.1007/s00438-013-0741-0

M3 - Review

C2 - 23539154

VL - 288

SP - 207

EP - 229

JO - Molecular Genetics and Genomics

JF - Molecular Genetics and Genomics

SN - 1617-4615

IS - 5-6

ER -