Implication of

CAKUT FOXD2 PAX2 WNT4 chronic kidney disease renal hypoplasia

Journal

medRxiv : the preprint server for health sciences
Titre abrégé: medRxiv
Pays: United States
ID NLM: 101767986

Informations de publication

Date de publication:
22 Mar 2023
Historique:
pubmed: 31 3 2023
medline: 31 3 2023
entrez: 30 3 2023
Statut: epublish

Résumé

Congenital anomalies of the kidney and urinary tract (CAKUT) are the predominant cause for chronic kidney disease below 30 years of age. Many monogenic forms have been discovered mainly due to comprehensive genetic testing like exome sequencing (ES). However, disease-causing variants in known disease-associated genes still only explain a proportion of cases. Aim of this study was to unravel the underlying molecular mechanism of syndromic CAKUT in two multiplex families with presumed autosomal recessive inheritance. ES in the index individuals revealed two different rare homozygous variants in In summary, our data implicate that

Sections du résumé

Background UNASSIGNED
Congenital anomalies of the kidney and urinary tract (CAKUT) are the predominant cause for chronic kidney disease below 30 years of age. Many monogenic forms have been discovered mainly due to comprehensive genetic testing like exome sequencing (ES). However, disease-causing variants in known disease-associated genes still only explain a proportion of cases. Aim of this study was to unravel the underlying molecular mechanism of syndromic CAKUT in two multiplex families with presumed autosomal recessive inheritance.
Methods and Results UNASSIGNED
ES in the index individuals revealed two different rare homozygous variants in
Conclusions UNASSIGNED
In summary, our data implicate that

Identifiants

pubmed: 36993625
doi: 10.1101/2023.03.21.23287206
pmc: PMC10055578
pii:
doi:

Types de publication

Preprint

Langues

eng

Déclaration de conflit d'intérêts

Competing interests The authors declare no competing interests.

Auteurs

Korbinian M Riedhammer (KM)

Institute of Human Genetics, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Munich, 81675, Germany.
Department of Nephrology, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Munich, 81675, Germany.

Thanh-Minh T Nguyen (TT)

Department of Human Genetics, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, 6525, The Netherlands.

Can Koşukcu (C)

Department of Bioinformatics, Hacettepe University Institute of Health Sciences, Ankara, 06100, Türkiye.

Julia Calzada-Wack (J)

Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, 85764, Germany.

Yong Li (Y)

Institute of Genetic Epidemiology, Faculty of Medicine and University Medical Center Freiburg, 79106 Freiburg, Germany.

Seha Saygılı (S)

Department of Pediatric Nephrology, Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Istanbul, Türkiye.

Vera Wimmers (V)

Institute of Experimental and Clinical Pharmacology and Toxicology II, Faculty of Medicine, University of Freiburg and, BIOSS Centre of Biological Signalling Studies, Albert-Ludwigs-University, Freiburg, 79104, Germany.
Center for Pediatrics and Adolescent Medicine, University Hospital Freiburg, Freiburg University Faculty of Medicine, Freiburg, 79106, Germany.

Gwang-Jin Kim (GJ)

Institute of Experimental and Clinical Pharmacology and Toxicology II, Faculty of Medicine, University of Freiburg and, BIOSS Centre of Biological Signalling Studies, Albert-Ludwigs-University, Freiburg, 79104, Germany.

Marialena Chrysanthou (M)

Department of Human Genetics, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, 6525, The Netherlands.

Zeineb Bakey (Z)

Department of Human Genetics, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, 6525, The Netherlands.
Center for Pediatrics and Adolescent Medicine, University Hospital Freiburg, Freiburg University Faculty of Medicine, Freiburg, 79106, Germany.

Markus Kraiger (M)

Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, 85764, Germany.

Adrián Sanz-Moreno (A)

Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, 85764, Germany.

Oana V Amarie (OV)

Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, 85764, Germany.

Birgit Rathkolb (B)

Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, 85764, Germany.
Institute of Molecular Animal Breeding and Biotechnology, Gene Center, Ludwig-Maximilians-University Munich, Munich, 81377, Germany.
German Center for Diabetes Research (DZD), Neuherberg, 85764, Germany.

Tanja Klein-Rodewald (T)

Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, 85764, Germany.

Lillian Garrett (L)

Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, 85764, Germany.
Institute of Developmental Genetics, Helmholtz Zentrum Munchen, German Research Center for Environmental Health, Neuherberg, 85764, Germany.

Sabine M Hölter (SM)

Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, 85764, Germany.
Institute of Developmental Genetics, Helmholtz Zentrum Munchen, German Research Center for Environmental Health, Neuherberg, 85764, Germany.
Chair of Developmental Genetics, TUM School of Life Sciences (SoLS), Technical University of Munich, Freising, 85354, Germany.

Claudia Seisenberger (C)

Institute of Molecular Animal Breeding and Biotechnology, Gene Center, Ludwig-Maximilians-University Munich, Munich, 81377, Germany.

Stefan Haug (S)

Institute of Genetic Epidemiology, Faculty of Medicine and University Medical Center Freiburg, 79106 Freiburg, Germany.

Susan Marschall (S)

Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, 85764, Germany.

Wolfgang Wurst (W)

Institute of Developmental Genetics, Helmholtz Zentrum Munchen, German Research Center for Environmental Health, Neuherberg, 85764, Germany.
Chair of Developmental Genetics, TUM School of Life Sciences (SoLS), Technical University of Munich, Freising, 85354, Germany.
Deutsches Institut fur Neurodegenerative Erkrankungen (DZNE) Site Munich, Munich, 81377, Germany.
Munich Cluster for Systems Neurology (SyNergy), Adolf-Butenandt-Institut, Ludwig-Maximilians-University Munich, Munich, 81377, Germany.

Helmut Fuchs (H)

Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, 85764, Germany.

Valerie Gailus-Durner (V)

Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, 85764, Germany.

Matthias Wuttke (M)

Institute of Genetic Epidemiology, Faculty of Medicine and University Medical Center Freiburg, 79106 Freiburg, Germany.

Martin Hrabe de Angelis (MH)

Institute of Experimental Genetics, German Mouse Clinic, Helmholtz Zentrum München, German Research Center for Environmental Health, Neuherberg, 85764, Germany.
German Center for Diabetes Research (DZD), Neuherberg, 85764, Germany.
Chair of Experimental Genetics, TUM School of Life Sciences (SoLS), Technical University of Munich, Freising, 85354, Germany.

Jasmina Ćomić (J)

Institute of Human Genetics, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Munich, 81675, Germany.
Department of Nephrology, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Munich, 81675, Germany.

Özlem Akgün Doğan (ÖA)

Department of Pediatric Genetics, Acibadem Mehmet Ali Aydinlar University, Faculty of Medicine, Istanbul, Türkiye.

Yasemin Özlük (Y)

Department of Pathology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Türkiye.

Mehmet Taşdemir (M)

Department of Pediatric Nephrology, Istinye University School of Medicine, Liv Hospital, Istanbul, Türkiye.

Ayşe Ağbaş (A)

Department of Pediatric Nephrology, Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Istanbul, Türkiye.

Nur Canpolat (N)

Department of Pediatric Nephrology, Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Istanbul, Türkiye.

Salim Ćalışkan (S)

Department of Pediatric Nephrology, Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Istanbul, Türkiye.

Ruthild Weber (R)

Department of Human Genetics, Hannover Medical School, Hannover, 30625, Germany.

Carsten Bergmann (C)

Medizinische Genetik Mainz, Limbach Genetics, Mainz, Germany.
Department of Medicine IV, Faculty of Medicine, Medical Center-University of Freiburg, Freiburg, Germany.

Cecile Jeanpierre (C)

Inserm U1163, Laboratoire des Maladies Renales Hereditaires Institut Imagine, Université de Paris, Paris, France.

Sophie Saunier (S)

Inserm U1163, Laboratoire des Maladies Renales Hereditaires Institut Imagine, Université de Paris, Paris, France.

Tze Y Lim (TY)

Department of Medicine, Division of Nephrology, Columbia University, New York, New York, USA.

Friedhelm Hildebrandt (F)

Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, MA, USA.

Bader Alhaddad (B)

Institute of Human Genetics, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Munich, 81675, Germany.

Kaman Wu (K)

Department of Human Genetics, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, 6525, The Netherlands.

Dinu Antony (D)

Department of Human Genetics, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, 6525, The Netherlands.
Institute of Experimental and Clinical Pharmacology and Toxicology II, Faculty of Medicine, University of Freiburg and, BIOSS Centre of Biological Signalling Studies, Albert-Ludwigs-University, Freiburg, 79104, Germany.

Julia Matschkal (J)

Department of Nephrology, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Munich, 81675, Germany.

Christian Schaaf (C)

Institute of Human Genetics, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Munich, 81675, Germany.
Department of Nephrology, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Munich, 81675, Germany.

Lutz Renders (L)

Department of Nephrology, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Munich, 81675, Germany.

Christoph Schmaderer (C)

Department of Nephrology, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Munich, 81675, Germany.

Thomas Meitinger (T)

Institute of Human Genetics, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Munich, 81675, Germany.

Uwe Heemann (U)

Department of Nephrology, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Munich, 81675, Germany.

Anna Köttgen (A)

Institute of Genetic Epidemiology, Faculty of Medicine and University Medical Center Freiburg, 79106 Freiburg, Germany.
CIBSS - Center for Integrative Biological Signaling Studies, University of Freiburg, 79106 Freiburg, Germany.

Sebastian Arnold (S)

Institute of Experimental and Clinical Pharmacology and Toxicology II, Faculty of Medicine, University of Freiburg and, BIOSS Centre of Biological Signalling Studies, Albert-Ludwigs-University, Freiburg, 79104, Germany.
CIBSS - Center for Integrative Biological Signaling Studies, University of Freiburg, 79106 Freiburg, Germany.

Fatih Ozaltin (F)

Department of Bioinformatics, Hacettepe University Institute of Health Sciences, Ankara, 06100, Türkiye.
Department of Pediatric Nephrology, Hacettepe University Faculty of Medicine, 06100, Sihhiye, Ankara, Türkiye.

Miriam Schmidts (M)

Department of Human Genetics, Radboud Institute for Molecular Life Sciences, Radboud University Medical Center, Nijmegen, 6525, The Netherlands.
Center for Pediatrics and Adolescent Medicine, University Hospital Freiburg, Freiburg University Faculty of Medicine, Freiburg, 79106, Germany.
CIBSS - Center for Integrative Biological Signaling Studies, University of Freiburg, 79106 Freiburg, Germany.

Julia Hoefele (J)

Institute of Human Genetics, Klinikum rechts der Isar, Technical University of Munich, School of Medicine, Munich, 81675, Germany.

Classifications MeSH