Human


Journal

eLife
ISSN: 2050-084X
Titre abrégé: Elife
Pays: England
ID NLM: 101579614

Informations de publication

Date de publication:
11 Jul 2024
Historique:
medline: 11 7 2024
pubmed: 11 7 2024
entrez: 11 7 2024
Statut: epublish

Résumé

Rare early-onset lower urinary tract disorders include defects of functional maturation of the bladder. Current treatments do not target the primary pathobiology of these diseases. Some have a monogenic basis, such as urofacial, or Ochoa, syndrome (UFS). Here, the bladder does not empty fully because of incomplete relaxation of its outflow tract, and subsequent urosepsis can cause kidney failure. UFS is associated with biallelic variants of

Identifiants

pubmed: 38990208
doi: 10.7554/eLife.91828
pii: 91828
doi:
pii:

Substances chimiques

Glucuronidase EC 3.2.1.31
heparanase EC 3.2.1.-

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Subventions

Organisme : Medical Research Council
ID : MR/L002744/1
Pays : United Kingdom
Organisme : Medical Research Council
ID : MR/T016809/1
Pays : United Kingdom
Organisme : Medical Research Council
ID : DTP
Pays : United Kingdom
Organisme : Kidney Research UK
ID : Paed_RP/002/20190925
Organisme : Kidney Research UK
ID : Paed_RP/005/20190925
Organisme : Newlife - The Charity for Disabled Children
ID : 15-15/03
Organisme : Newlife - The Charity for Disabled Children
ID : 15-16/06
Organisme : Manchester Biomedical Research Centre
ID : IS-BRC-1215-20007
Organisme : Kidneys for Life
ID : start-up grant 2018
Organisme : Kidneys for Life
ID : project grant 2023
Organisme : LifeArc
ID : Pathfinder Award
Organisme : Medical Research Council
ID : Rare Disease Research Platform MR/Y008340/1
Pays : United Kingdom

Informations de copyright

© 2024, Lopes et al.

Déclaration de conflit d'intérêts

FL, CG, BJ, SA, AL, AG, WN, AW, NR No competing interests declared, SW is a co-founder of Bloomsbury Genetic Therapies and is a member of the SMAB of Forge Biologics

Auteurs

Filipa M Lopes (FM)

Division of Cell Matrix Biology and Regenerative Medicine, School of Biological Sciences, Faculty of Biology Medicine and Health, University of Manchester, Manchester, United Kingdom.

Celine Grenier (C)

Division of Cell Matrix Biology and Regenerative Medicine, School of Biological Sciences, Faculty of Biology Medicine and Health, University of Manchester, Manchester, United Kingdom.

Benjamin W Jarvis (BW)

Division of Cell Matrix Biology and Regenerative Medicine, School of Biological Sciences, Faculty of Biology Medicine and Health, University of Manchester, Manchester, United Kingdom.

Sara Al Mahdy (S)

Division of Cell Matrix Biology and Regenerative Medicine, School of Biological Sciences, Faculty of Biology Medicine and Health, University of Manchester, Manchester, United Kingdom.

Adrian Lène-McKay (A)

Division of Cell Matrix Biology and Regenerative Medicine, School of Biological Sciences, Faculty of Biology Medicine and Health, University of Manchester, Manchester, United Kingdom.

Alison M Gurney (AM)

Division of Pharmacy and Optometry, School of Health Sciences, Faculty of Biology Medicine and Health, University of Manchester, Manchester, United Kingdom.

William G Newman (WG)

Manchester Centre for Genomic Medicine, Manchester University NHS Foundation Trust, Manchester Academic Health Science Centre, Manchester, United Kingdom.
Division of Evolution Infection and Genomics, School of Biological Sciences, Faculty of Biology Medicine and Health, University of Manchester, Manchester, United Kingdom.

Simon N Waddington (SN)

Maternal & Fetal Medicine, EGA Institute for Women's Health, Faculty of Population Health Sciences, University College London, London, United Kingdom.
Wits/SAMRC Antiviral Gene Therapy Research Unit, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa.

Adrian S Woolf (AS)

Division of Cell Matrix Biology and Regenerative Medicine, School of Biological Sciences, Faculty of Biology Medicine and Health, University of Manchester, Manchester, United Kingdom.

Neil A Roberts (NA)

Division of Cell Matrix Biology and Regenerative Medicine, School of Biological Sciences, Faculty of Biology Medicine and Health, University of Manchester, Manchester, United Kingdom.

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Classifications MeSH