Document Detail

Mechanisms of apoptosis in retinitis pigmentosa.
MedLine Citation:
PMID:  19355918     Owner:  NLM     Status:  MEDLINE    
Mutations in humans are associated with several forms of inherited retinal dystrophies, such as Retinitis Pigmentosa which lead to retinal cell death and irreversible loss of vision. Genes involved in affected patients mainly encode proteins related to vision physiology including visual cycle and light-dependent phototransduction cascade. As reported in spontaneous and genetically engineered mouse models, apoptosis is a common fate in retinal degeneration, although the triggered signals to retinal apoptosis remain largely unraveled. Several studies highlighted that many of the molecular pathways involved in ocular diseases rely on caspase-dependent or -independent apoptotic mitochondrial pathway involving the Bcl-2 family of proteins. Anti- and pro-apoptotic Bcl-2 members are present in retinal tissues and are thought to play a role in the pathogenesis of several retinal disorders. Since almost no efficient treatments are available so far, it remains a great challenge to decipher the molecular pathways involved in retinal dystrophies and to develop alternative therapies to prevent or inhibit eye defect. Toward this goal, mutation-independent strategies such as molecular therapy provides promising and exciting approaches to deliver anti-apoptotic molecules targeting the Bcl-2 pathway through the use of cell permeable transport peptides. Modulation of common apoptotic signaling pathways may be of outstanding potential to target multiple retinal dystrophies regardless of the primary genetic defect.
Sandra Cottet; Daniel F Schorderet
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Publication Detail:
Type:  Journal Article; Research Support, Non-U.S. Gov't; Review    
Journal Detail:
Title:  Current molecular medicine     Volume:  9     ISSN:  1566-5240     ISO Abbreviation:  Curr. Mol. Med.     Publication Date:  2009 Apr 
Date Detail:
Created Date:  2009-04-09     Completed Date:  2009-05-26     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101093076     Medline TA:  Curr Mol Med     Country:  Netherlands    
Other Details:
Languages:  eng     Pagination:  375-83     Citation Subset:  IM    
IRO, Institute for Research in Ophthalmology, Sion, Switzerland.
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MeSH Terms
Apoptosis / physiology*
Caspases / metabolism
Light Signal Transduction / physiology
Retinitis Pigmentosa / genetics,  pathology*,  physiopathology*
Signal Transduction / physiology
Reg. No./Substance:
EC 3.4.22.-/Caspases

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

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