Photoreceptor Cell Death Mechanisms in Inherited Retinal Degeneration

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Photoreceptor cell death is the major hallmark of a group of human inherited retinal degenerations commonly referred to as retinitis pigmentosa (RP). Although the causative genetic mutations are often known, the mechanisms leading to photoreceptor degeneration remain poorly defined. Previous research work has focused on apoptosis, but recent evidence suggests that photoreceptor cell death may result primarily from non-apoptotic mechanisms independently of AP1 or p53 transcription factor activity, Bcl proteins, caspases, or cytochrome c release. This review briefly describes some animal models used for studies of retinal degeneration, with particular focus on the rd1 mouse. After outlining the major features of different cell death mechanisms in general, we then compare them with results obtained in retinal degeneration models, where photoreceptor cell death appears to be governed by, among other things, changes in cyclic nucleotide metabolism, downregulation of the transcription factor CREB, and excessive activation of calpain and PARP. Based on recent experimental evidence, we propose a putative non-apoptotic molecular pathway for photoreceptor cell death in the rd1 retina. The notion that inherited photoreceptor cell death is driven by non-apoptotic mechanisms may provide new ideas for future treatment of RP.

MEDICIN OCH HÄLSOVETENSKAP Medicinska och farmaceutiska grundvetenskaper Neurovetenskaper hsv//swe
MEDICAL AND HEALTH SCIENCES Basic Medicine Neurosciences hsv//eng
Retina
rd1
PARP
CREB
Oxidative stress
Calpain
cGMP
AIF
Calcium
rd2
rds
APOPTOSIS-INDUCING FACTOR
ROD CGMP-PHOSPHODIESTERASE
ENDOPLASMIC-RETICULUM STRESS
ELEMENT-BINDING PROTEIN
CAMP EARLY
REPRESSOR
RD1 MOUSE RETINA
MEDIATED PROGRAMMED NECROSIS
CALCIUM-CHANNEL BLOCKER
NITRIC-OXIDE SYNTHASE
D-CIS-DILTIAZEM

Arango-Gonzalez, B. (author)
Kustermann, S. (author)
Romero, F. J. (author)
van Veen, TheoLund University,Lunds universitet,Oftalmologi, Lund,Sektion IV,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Ophthalmology, Lund,Section IV,Department of Clinical Sciences, Lund,Faculty of Medicine(Swepub:lu)oft-tve (author)
Zrenner, E. (author)
Ekström, PerLund University,Lunds universitet,Oftalmologi, Lund,Sektion IV,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Ophthalmology, Lund,Section IV,Department of Clinical Sciences, Lund,Faculty of Medicine(Swepub:lu)zoof-pek (author)
Paquet-Durand, F. (author)
Oftalmologi, LundSektion IV (creator_code:org_t)

In:Molecular Neurobiology: Springer Science and Business Media LLC38:3, s. 253-2691559-11820893-7648

By the author/editor: Sancho-Pelluz, J ...; Arango-Gonzalez, ...; Kustermann, S.; Romero, F. J.; van Veen, Theo; Zrenner, E.; show more...; Ekström, Per; Paquet-Durand, F ...; show less...

About the subject

MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Basic Medicine; and Neurosciences

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