Document Detail


Cause of progression in Duchenne muscular dystrophy: impaired differentiation more probable than replicative aging.
MedLine Citation:
PMID:  11521207     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Replicative aging of myogenic cells (satellite cells) owing to enhanced myofiber turnover is a common explanation of the progression of Duchenne muscular dystrophy (DMD). This hypothesis has been reexamined recently by telomere length measurements in dystrophic tissue. We evaluate the controversial results of these studies. We also review a large body of in vitro, animal (mdx), and patient data which indicate that impaired differentiation, but not replicative aging, is the leading cause of progression in DMD. We recommend in vivo investigations of cell kinetics in DMD muscle, as well as telomere length and telomerase analyses of DMD satellite cells in vitro for a definite judgement of the replicative aging hypothesis. Analogous investigations were helpful in AIDS research where replicative aging was embraced as a simple explanation of the paradigmatic CD4 lymphocyte decline but had to be rejected in favour of more complex models of disturbed lymphocyte homeostasis and regeneration. The question of replicative aging versus impaired differentiation is relevant for the understanding of therapeutic failures and the design of new strategies. Impaired differentiation is compatible with the failure of myoblast transfer in DMD and calls for further studies on the myofiber environment. Replicative aging, on the other hand, could possibly be treated by telomerase gene delivery.
Authors:
K Oexle; A Kohlschütter
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Neuropediatrics     Volume:  32     ISSN:  0174-304X     ISO Abbreviation:  Neuropediatrics     Publication Date:  2001 Jun 
Date Detail:
Created Date:  2001-08-24     Completed Date:  2001-10-11     Revised Date:  2008-01-16    
Medline Journal Info:
Nlm Unique ID:  8101187     Medline TA:  Neuropediatrics     Country:  Germany    
Other Details:
Languages:  eng     Pagination:  123-9     Citation Subset:  IM    
Affiliation:
Abteilung für Stoffwechsel und Molekulare Pädiatrie, Kinderspital, Universität Zürich, Switzerland. konrad.oexle@kispi.unizh.ch
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MeSH Terms
Descriptor/Qualifier:
Acquired Immunodeficiency Syndrome / pathology
Animals
CD4-Positive T-Lymphocytes / pathology
Cell Aging / physiology*
Cell Differentiation / physiology*
Cell Division / physiology*
Child
Disease Progression
Humans
Mice
Mice, Inbred mdx
Muscle, Skeletal / pathology
Muscular Dystrophy, Animal / pathology
Muscular Dystrophy, Duchenne / pathology*
Regeneration / physiology
Telomere / pathology

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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