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Klin Monbl Augenheilkd 2000; 216(2): 83-89
DOI: 10.1055/s-2000-10523
ÜBERSICHT

Georg Thieme Verlag Stuttgart · New York

Therapieversuche bei RP: Licht am Ende des Tunnels?[1] [2]

Therapeutic Approaches in RP: Light at the End of the Tunnel?Mathias Abegg, Farhad Hafezi, Andreas Wenzel, Christian Grimm, Charlotte  E. Remé
  • Labor für Zellbiologie der Netzhaut, Universitäts-Augenklinik Zürich (Direktor: Prof. Dr. B. Gloor), Frauenklinikstrasse 24,
  • CH-8091 Zürich
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Publication History

Publication Date:
31 December 2000 (online)

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Zusammenfassung

Die genetisch und klinisch heterogene Netzhauterkrankung Retinitis Pigmentosa stellt mit einer Prävalenz von 3,5 ‰ eine relativ häufige Ursache für Erblindung in den Industrienationen dar. Seit Jahrzehnten wird an einer Vielzahl von Tiermodellen, die zum Teil dem Menschen analoge Mutationen tragen, die Ätiologie dieser Krankheit studiert. Es steht jedoch bis heute keine Therapie zur Verfügung, die den Verlauf dieser Erkrankung beeinflussen und den apoptotischen Zelltod der Photorezeptoren aufhalten könnte. In letzter Zeit haben sich vielversprechende neue Therapieansätze ergeben, die nun im Tiermodell erprobt werden. Neben Versuchen, die degenerierten Photorezeptorzellen durch einen prä- oder subretinal eingepflanzten Mikrochip zu ersetzen, scheinen sowohl die somatische Gentherapie als auch die Hemmung des Photorezeptorzelltodes durch Apoptose mittels Wachstumsfaktoren und anderen pharmakologischen Substanzen aussichtsreiche Ansätze für künftige Therapien zu sein. Ein besseres Verständnis der molekularen Abläufe, die der Degeneration der Photorezeptorzellen durch Apoptose zugrunde liegen, ist dabei jedoch eine unabdingbare Voraussetzung.

Retinitis pigmentosa (RP) is a hereditary retinal dystrophy which leads to severe visual impairment or blindness and affects about 3.5 ‰ of individuals in the industrial world. During the past decades, numerous animal models carrying mutations analogous to mutations in human RP have been studied to elucidate the molecular mechanisms leading to apoptotic photoreceptor cell death in this disease. Up to date, there is no effective treatment to influence the fatal outcome of RP. Recent progress in basic research promotes the development of new therapeutic strategies. In order to restore visual function in blind individuals, the development of electronic photoreceptor prosthesis is being investigated by several researchgroups. Other promising approaches are somatic gene therapy, the application of growth factors and/or pharmacological agents and the inhibition of photoreceptor cell death by interfering with the apoptotic pathway. However, a better understanding of the molecular events leading to cell loss due to photoreceptor apoptosis will be essential for the development of effective treatment.

Schlüsselwörter

rd - Retinitis Pigmentosa - Netzhautdystrophie - Apoptose - c-fos

Key words

rd - retinitis pigmentosa - retinal dystrophy - apoptosis - c-fos

1 Herrn Prof. Dr. Balder Gloor gewidmet.

2 Manuskript eingereicht am 18. 8. 99 und in der vorliegenden Form angenommen.

Literatur

1 Herrn Prof. Dr. Balder Gloor gewidmet.

2 Manuskript eingereicht am 18. 8. 99 und in der vorliegenden Form angenommen.

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