Should Patients with Kearns-Sayre Syndrome and Corneal Endothelial Failure Be Genotyped for a


Journal

Genes
ISSN: 2073-4425
Titre abrégé: Genes (Basel)
Pays: Switzerland
ID NLM: 101551097

Informations de publication

Date de publication:
29 11 2021
Historique:
received: 17 10 2021
revised: 23 11 2021
accepted: 28 11 2021
entrez: 24 12 2021
pubmed: 25 12 2021
medline: 17 2 2022
Statut: epublish

Résumé

The aim of this study was to describe the ocular phenotype in a case with Kearns-Sayre syndrome (KSS) spectrum and to determine if corneal endothelial cell dysfunction could be attributed to other known distinct genetic causes. Herein, genomic DNA was extracted from blood and exome sequencing was performed. Non-coding gene regions implicated in corneal endothelial dystrophies were screened by Sanger sequencing. In addition, a repeat expansion situated within an intron of

Identifiants

pubmed: 34946867
pii: genes12121918
doi: 10.3390/genes12121918
pmc: PMC8702069
pii:
doi:

Substances chimiques

DNA, Mitochondrial 0
TCF4 protein, human 0
Transcription Factor 4 0

Types de publication

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

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : Medical Research Council
ID : MR/S031820/1
Pays : United Kingdom

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Auteurs

Lubica Dudakova (L)

Department of Paediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University and General University Hospital in Prague, 128 08 Prague, Czech Republic.

Pavlina Skalicka (P)

Department of Paediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University and General University Hospital in Prague, 128 08 Prague, Czech Republic.
Department of Ophthalmology, First Faculty of Medicine, Charles University and General University Hospital in Prague, 128 08 Prague, Czech Republic.

Alice E Davidson (AE)

UCL Institute of Ophthalmology, London EC1V 9EL, UK.
Moorfields Eye Hospital, London EC1V 2PD, UK.

Amanda N Sadan (AN)

UCL Institute of Ophthalmology, London EC1V 9EL, UK.

Monika Chylova (M)

Department of Paediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University and General University Hospital in Prague, 128 08 Prague, Czech Republic.

Helena Jahnova (H)

Department of Paediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University and General University Hospital in Prague, 128 08 Prague, Czech Republic.

Nicole Anteneova (N)

Department of Paediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University and General University Hospital in Prague, 128 08 Prague, Czech Republic.

Marketa Tesarova (M)

Department of Paediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University and General University Hospital in Prague, 128 08 Prague, Czech Republic.

Tomas Honzik (T)

Department of Paediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University and General University Hospital in Prague, 128 08 Prague, Czech Republic.

Petra Liskova (P)

Department of Paediatrics and Inherited Metabolic Disorders, First Faculty of Medicine, Charles University and General University Hospital in Prague, 128 08 Prague, Czech Republic.
Department of Ophthalmology, First Faculty of Medicine, Charles University and General University Hospital in Prague, 128 08 Prague, Czech Republic.
UCL Institute of Ophthalmology, London EC1V 9EL, UK.

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Classifications MeSH