Authors: Rebolledo, Daniela¹; Minniti, Alicia¹; Grez, Paula¹; Fadic, Ricardo²; Kohn, Rebecca³; Inestrosa, Nibaldo⁴

Source: Molecular Neurobiology, Volume 38, Number 2, October 2008 , pp. 178-198(21)

Publisher: Humana Press

Abstract:

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.

Keywords: IBM; Inclusion Body Myositis; Muscle; C. elegans; Aβ peptide; Myopathy; Invertebrate models

Document Type: Research article

DOI: http://dx.doi.org/10.1007/s12035-008-8041-0

Affiliations: 1: Centro de Envejecimiento y Regeneración (CARE), Centro de Regulación Celular y Patología “Joaquín V. Luco” (CRCP), MIFAB, Facultad de Ciencias Biológicas, P. Universidad Católica de Chile, Alameda, 340, Santiago, Chile, 2: Departamento de Neurología, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile, 3: Department of Biology, Ursinus College, Collegeville, PA, USA, 4: Centro de Envejecimiento y Regeneración (CARE), Centro de Regulación Celular y Patología “Joaquín V. Luco” (CRCP), MIFAB, Facultad de Ciencias Biológicas, P. Universidad Católica de Chile, Alameda, 340, Santiago, Chile, Email: ninestrosa@bio.puc.cl

Publication date: 2008-10-01

Related content

• In this: publication
• By this: publisher
• In this Subject: Anatomy & Physiology ,  Zoology
• By this author: Rebolledo, Daniela ;  Minniti, Alicia ;  Grez, Paula ;  Fadic, Ricardo ;  Kohn, Rebecca ;  Inestrosa, Nibaldo

Website © Publishing Technology. Article copyright remains with the publisher, society or author(s) as specified within the article.

• Terms and conditions
• Privacy policy
• Information for advertisers