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- Ward, RebeccaUniv Coll Dublin, Ireland (author)
Pharmacological restoration of visual function in a zebrafish model of von-Hippel Lindau disease
- Article/chapterEnglish2020
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- ACADEMIC PRESS INC ELSEVIER SCIENCE,2020
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- LIBRIS-ID:oai:DiVA.org:liu-164050
- https://urn.kb.se/resolve?urn=urn:nbn:se:liu:diva-164050URI
- https://doi.org/10.1016/j.ydbio.2019.02.008DOI
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- Language:English
- Summary in:English
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- Funding Agencies|Fighting Blindness MRCG/HRB [MRCG-2014-3]; Irish Research Council/Fighting Blindness [EPSPG/2017/276]; European UnionEuropean Union (EU) [734907]
- Von Hippel-Lindau (VHL) syndrome is a rare, autosomal dominant disorder, characterised by hypervascularised tumour formation in multiple organ systems. Vision loss associated with retinal capillary hemangioblastomas remains one of the earliest complications of VHL disease. The mortality of Vhl(-/-) mice in utero restricted modelling of VHL disease in this mammalian model. Zebrafish harbouring a recessive germline mutation in the vhl gene represent a viable, alternative vertebrate model to investigate associated ocular loss-of-function phenotypes. Previous studies reported neovascularisation of the brain, eye and trunk together with oedema in the vhl(-/-) zebrafish eye. In this study, we demonstrate vhl(-/-) zebrafish almost entirely lack visual function. Furthermore, hyaloid vasculature networks in the vhl(-/-) eye are improperly formed and this phenotype is concomitant with development of an ectopic intraretinal vasculature. Sunitinib malate, a multi tyrosine kinase inhibitor, market authorised for cancer, reversed the ocular behavioural and morphological phenotypes observed in vhl(-/-) zebrafish. We conclude that the zebrafish yid gene contributes to an endogenous molecular barrier that prevents development of intraretinal vasculature, and that pharmacological intervention with sunitinib can improve visual function and hyaloid vessel patterning while reducing abnormally formed ectopic intraretinal vessels in vhl(-/-) zebrafish.
Subject headings and genre
- NATURVETENSKAP Biologi Utvecklingsbiologi hsv//swe
- NATURAL SCIENCES Biological Sciences Developmental Biology hsv//eng
- Von Hippel-Lindau; VHL; Retinal neovascularisation; Zebrafish; Sunitinib malate
Added entries (persons, corporate bodies, meetings, titles ...)
- Ali, ZaheerLinköpings universitet,Avdelningen för kardiovaskulär medicin,Medicinska fakulteten(Swepub:liu)aliza36 (author)
- Slater, KayleighUniv Coll Dublin, Ireland (author)
- Reynolds, Alison L.Univ Coll Dublin, Ireland; Univ Coll Dublin, Ireland (author)
- Jensen, LasseLinköpings universitet,Avdelningen för diagnostik och specialistmedicin,Medicinska fakulteten,Region Östergötland, Klinisk farmakologi(Swepub:liu)larje86 (author)
- Kennedy, Breandan N.Univ Coll Dublin, Ireland (author)
- Univ Coll Dublin, IrelandAvdelningen för kardiovaskulär medicin (creator_code:org_t)
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- In:Developmental Biology: ACADEMIC PRESS INC ELSEVIER SCIENCE457:2, s. 226-2340012-16061095-564X
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