Journal
NEURODEGENERATIVE DISEASES
Volume 2, Issue 3-4, Pages 185-194Publisher
KARGER
DOI: 10.1159/000089624
Keywords
Reticulons; Reticulons as disease markers; Mammalian reticulons; Mammalian reticulons, structure; Mammalian reticulons, functions
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Funding
- Association pour la Recherche sur la Sclerose Laterale Amyotrophique (ARS)
- Association pour la Recherche contre les Maladies Neurodegeneratives (AREMANE)
- Region-Alsace
- Association Francaise contre les Myopathies (AFM)
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Reticulons (RTNs) are a family of proteins that are primarily associated with the endoplasmic reticulum. In mammals, four genes have been identified and referred as to rtn1, 2, 3 and the neurite outgrowth inhibitor rtn4/nogo. These genes generate multiple isoforms that contain a common C-terminal reticulon homology domain of 150-200 amino-acid residues. The N-terminal regions of RTNs are highly variable, and result from alternative splicing or differential promoter usage. Although widely distributed, the functions of RTNs are still poorly understood. Much interest has been focused on rtn4/nogo because of its activity as a potent inhibitor of axonal growth and repair. In the present study, we update recent knowledge on mammalian RTNs paying special attention to the involvement of these proteins as markers of neurological diseases. We also present recent data concerning RTN expression in amyotrophic lateral sclerosis, a fatal degenerative disorder characterized by loss of upper and lower motor neurons, and muscle atrophy. The rearrangement of RTN expression is regulated not only in suffering skeletal muscle but also preceding the onset of symptoms, and may relate to the disease process. Copyright (C) 2005 S. Karger AG, Basel
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