Molecular biology of amyotrophic lateral sclerosis: insights from genetics.

Pasinelli P; Brown RH

doi:10.1038/nrn1971

Molecular biology of amyotrophic lateral sclerosis: insights from genetics.

Pasinelli P ¹,

Brown RH

Affiliations

1. Day Neuromuscular Research Laboratory, Massachusetts General Hospital, Room 3125, Building 114, 16th Street, Navy Yard, Charlestown, Massachusetts 02429, USA.
Authors
Pasinelli P¹
(1 author)

ORCIDs linked to this article

Pasinelli P | 0000-0002-3385-2707

Nature reviews. Neuroscience, 01 Sep 2006, 7(9):710-723
https://doi.org/10.1038/nrn1971 PMID: 16924260

Review

Abstract

Amyotrophic lateral sclerosis (ALS) is a paralytic disorder caused by motor neuron degeneration. Mutations in more than 50 human genes cause diverse types of motor neuron pathology. Moreover, defects in five Mendelian genes lead to motor neuron disease, with two mutations reproducing the ALS phenotype. Analyses of these genetic effects have generated new insights into the diverse molecular pathways involved in ALS pathogenesis. Here, we present an overview of the mechanisms for motor neuron death and of the role of non-neuronal cells in ALS.

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References

Articles referenced by this article (214)

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MED: 11715057
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Search life-sciences literature (45,103,589 articles, preprints and more)

Molecular biology of amyotrophic lateral sclerosis: insights from genetics.

Affiliations

Authors

ORCIDs linked to this article

Abstract

Full text links

References

Mulder, D. W. Clinical limits of amyotrophic lateral sclerosis. Adv. Neurol. 36, 15–22 (1982).

Incidence of amyotrophic lateral sclerosis in three counties in western Washington state.

Mitsumoto, H., Chad, D. A. & Pioro, E. P. Amyotrophic Lateral Sclerosis (Oxford Univ. Press, New York, 1998).

Kurtzke, J. K. L. in Clinical Neurology (ed. Joynt, R.) (Lippincott, Philadelphia, 1989).

Kurtzke, J. F. Risk factors in amyotrophic lateral sclerosis. Adv. Neurol. 56, 245–270 (1991).

Ince, P. G. in Amyotrophic Lateral Sclerosis (eds Brown, R. H. Jr, Meininger, V. & Swash, M.) 83–112 (Martin Dunitz, London, 2000).

From Charcot to Lou Gehrig: deciphering selective motor neuron death in ALS.

Rowland, L. P. in Amyotrophic Lateral Sclerosis and Other Motor Neuron Diseases (ed. Rowland, L. P.) 3–23 (Raven, 1992).

An ALS-like syndrome with new HIV infection and complete response to antiretroviral therapy.

Detection of serum reverse transcriptase activity in patients with ALS and unaffected blood relatives.

Citations & impact

Impact metrics

Citations of article over time

Alternative metrics

Article citations

Ferritin is closely associated with microglia in amyotrophic lateral sclerosis.

Urolithin a Improves Motor Dysfunction Induced by Copper Exposure in SOD1<sup>G93A</sup> Transgenic Mice Via Activation of Mitophagy.

Significance of Programmed Cell Death Pathways in Neurodegenerative Diseases.

Klotho: molecular mechanisms and emerging therapeutics in central nervous system diseases.

AAV-NRIP gene therapy ameliorates motor neuron degeneration and muscle atrophy in ALS model mice.

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Search life-sciences literature (45,103,589 articles, preprints and more)

Molecular biology of amyotrophic lateral sclerosis: insights from genetics.

Author information

Affiliations

Authors

ORCIDs linked to this article

Abstract

Full text links

References

Mulder, D. W. Clinical limits of amyotrophic lateral sclerosis. Adv. Neurol. 36, 15–22 (1982).

Mitsumoto, H., Chad, D. A. & Pioro, E. P. Amyotrophic Lateral Sclerosis (Oxford Univ. Press, New York, 1998).

Kurtzke, J. K. L. in Clinical Neurology (ed. Joynt, R.) (Lippincott, Philadelphia, 1989).

Kurtzke, J. F. Risk factors in amyotrophic lateral sclerosis. Adv. Neurol. 56, 245–270 (1991).

Ince, P. G. in Amyotrophic Lateral Sclerosis (eds Brown, R. H. Jr, Meininger, V. & Swash, M.) 83–112 (Martin Dunitz, London, 2000).

Rowland, L. P. in Amyotrophic Lateral Sclerosis and Other Motor Neuron Diseases (ed. Rowland, L. P.) 3–23 (Raven, 1992).

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