RGD Reference Report - Expression of Bcl-2 protects against photoreceptor degeneration in retinal degeneration slow (rds) mice. - Rat Genome Database

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Expression of Bcl-2 protects against photoreceptor degeneration in retinal degeneration slow (rds) mice.

Authors: Nir, I  Kedzierski, W  Chen, J  Travis, GH 
Citation: Nir I, etal., J Neurosci. 2000 Mar 15;20(6):2150-4.
RGD ID: 8554863
Pubmed: PMID:10704489   (View Abstract at PubMed)
PMCID: PMC6772515   (View Article at PubMed Central)

The retinal degeneration slow or rds gene encodes rds/peripherin, an integral membrane glycoprotein in the outer segments of rod and cone photoreceptors. Mice homozygous for a null mutation in rds fail to develop outer segments and undergo subsequent degeneration of photoreceptors by the apoptotic pathway. Mutations in the human RDS gene are responsible for several forms of inherited blindness including autosomal-dominant retinitis pigmentosa and macular degeneration. Here, we examined the effects of ectopic Bcl-2 expression in transgenic photoreceptors on the rate of retinal degeneration in rds mutant mice. We observed an approximately twofold preservation of photoreceptors compared with nontransgenic rds mutant mice at 3 months. Immunoblot analysis showed similar levels of Bcl-2 in 2-, 3-, and 4-week-old transgenic mice. Expression of Bcl-2 in the rds mouse did not lead to outer segment formation and did not induce cell death. These results suggest that Bcl-2 expression may be an effective therapeutic strategy in humans with mutations in RDS or other genes that affect the integrity of photoreceptor outer segments.

RGD Manual Disease Annotations    Click to see Annotation Detail View
TermQualifierEvidenceWithReferenceNotesSourceOriginal Reference(s)
retinal degeneration treatmentIDA 8554863 RGD 
retinal degeneration treatmentISOBCL2 (Homo sapiens)8554863; 8554863 RGD 

Objects Annotated

Genes (Rattus norvegicus)
Bcl2  (BCL2, apoptosis regulator)

Genes (Mus musculus)
Bcl2  (B cell leukemia/lymphoma 2)

Genes (Homo sapiens)
BCL2  (BCL2 apoptosis regulator)


Additional Information