Inherited cataracts: molecular genetics, clinical features, disease mechanisms and novel therapeutic approaches.


Journal

The British journal of ophthalmology
ISSN: 1468-2079
Titre abrégé: Br J Ophthalmol
Pays: England
ID NLM: 0421041

Informations de publication

Date de publication:
10 2020
Historique:
received: 25 09 2019
revised: 20 11 2019
accepted: 28 01 2020
pubmed: 29 3 2020
medline: 20 2 2021
entrez: 29 3 2020
Statut: ppublish

Résumé

Cataract is the most common cause of blindness in the world; during infancy and early childhood, it frequently results in visual impairment. Congenital cataracts are phenotypically and genotypically heterogeneous and can occur in isolation or in association with other systemic disorders. Significant progress has been made in identifying the molecular genetic basis of cataract; 115 genes to date have been found to be associated with syndromic and non-syndromic cataract and 38 disease-causing genes have been identified to date to be associated with isolated cataract. In this review, we briefly discuss lens development and cataractogenesis, detail the variable cataract phenotypes and molecular mechanisms, including genotype-phenotype correlations, and explore future novel therapeutic avenues including cellular therapies and pharmacological treatments.

Identifiants

pubmed: 32217542
pii: bjophthalmol-2019-315282
doi: 10.1136/bjophthalmol-2019-315282
doi:

Substances chimiques

Aquaporins 0
Connexins 0
Crystallins 0
Cytoskeletal Proteins 0

Types de publication

Journal Article Research Support, Non-U.S. Gov't Review

Langues

eng

Sous-ensembles de citation

IM

Pagination

1331-1337

Subventions

Organisme : Wellcome Trust
ID : 099173/Z/12/Z
Pays : United Kingdom

Informations de copyright

© Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY. Published by BMJ.

Déclaration de conflit d'intérêts

Competing interests: None declared.

Auteurs

Vanita Berry (V)

Department of Genetics, UCL Institute of Ophthalmology, University College London, London, UK v.berry@ucl.ac.uk michel.michaelides@ucl.ac.uk.

Michalis Georgiou (M)

Department of Genetics, UCL Institute of Ophthalmology, University College London, London, UK.
Moorfields Eye Hospital NHS Foundation Trust, London, UK.

Kaoru Fujinami (K)

Department of Genetics, UCL Institute of Ophthalmology, University College London, London, UK.
National Institute of Sensory Organs, National Hospital Organization, Tokyo Medical Centre, Tokyo, Japan.

Roy Quinlan (R)

Department of Genetics, UCL Institute of Ophthalmology, University College London, London, UK.
Department of Biosciences, School of Biological and Medical Sciences, University of Durham, Durham, UK.

Anthony Moore (A)

Moorfields Eye Hospital NHS Foundation Trust, London, UK.
Ophthalmology Department, University of California School of Medicine, San Francisco, California, USA.

Michel Michaelides (M)

Department of Genetics, UCL Institute of Ophthalmology, University College London, London, UK v.berry@ucl.ac.uk michel.michaelides@ucl.ac.uk.
Moorfields Eye Hospital NHS Foundation Trust, London, UK.

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Classifications MeSH