Inclusion Body Myositis: A View from the Caenorhabditis elegans Muscle

Inclusion body myositis (IBM) is the most common myopathy in people over 50 years of age. It involves an inflammatory process that, paradoxically, does not respond to anti-inflammatory drugs. A key feature of IBM is the presence of amyloid-β-peptide aggregates called amyloid deposits, which are also...

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Veröffentlicht in:Molecular neurobiology 2008-10, Vol.38 (2), p.178-198
Hauptverfasser: Rebolledo, Daniela L., Minniti, Alicia N., Grez, Paula M., Fadic, Ricardo, Kohn, Rebecca, Inestrosa, Nibaldo C.
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container_end_page 198
container_issue 2
container_start_page 178
container_title Molecular neurobiology
container_volume 38
creator Rebolledo, Daniela L.
Minniti, Alicia N.
Grez, Paula M.
Fadic, Ricardo
Kohn, Rebecca
Inestrosa, Nibaldo C.
description Inclusion body myositis (IBM) is the most common myopathy in people over 50 years of age. It involves an inflammatory process that, paradoxically, does not respond to anti-inflammatory drugs. A key feature of IBM is the presence of amyloid-β-peptide aggregates called amyloid deposits, which are also characteristic of Alzheimer’s disease. The use of animals that mimic at least some characteristics of a disease has become very important in the quest to elucidate the molecular mechanisms underlying this and other pathogeneses. Although there are some transgenic mouse strains that recreate some aspects of IBM, in this review, we hypothesize that the great degree of similarity between nematode and human genes known to be involved in IBM as well as the considerable conservation of biological mechanisms across species is an important feature that must be taken into consideration when deciding on the use of this nematode as a model. Straightforward laboratory techniques (culture, transformation, gene knockdown, genetic screenings, etc.) as well as anatomical, physiological, and behavioral characteristics add to the value of this model. In the present work, we review evidence that supports the use of Caenorhabditis elegans as a biological model for IBM.
doi_str_mv 10.1007/s12035-008-8041-0
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subjects Amyloid beta-Peptides - genetics
Amyloid beta-Peptides - metabolism
Amyloid beta-Protein Precursor - genetics
Amyloid beta-Protein Precursor - metabolism
Animals
Biomedical and Life Sciences
Biomedicine
Caenorhabditis elegans
Caenorhabditis elegans - anatomy & histology
Caenorhabditis elegans - genetics
Cell Biology
Disease Models, Animal
Humans
Metals - metabolism
Mitochondria - metabolism
Muscles - metabolism
Muscles - pathology
Muscular system
Myositis, Inclusion Body - metabolism
Myositis, Inclusion Body - pathology
Nematoda
Neurobiology
Neurology
Neurosciences
Oxidative Stress
Peptides
Peptides - genetics
Peptides - metabolism
RNA Interference
Studies
Worms
title Inclusion Body Myositis: A View from the Caenorhabditis elegans Muscle
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