Recessive Mutations in

SYNPO2 monogenic kidney disease nephrotic syndrome

Journal

Kidney international reports
ISSN: 2468-0249
Titre abrégé: Kidney Int Rep
Pays: United States
ID NLM: 101684752

Informations de publication

Date de publication:
Feb 2021
Historique:
received: 05 09 2020
revised: 10 10 2020
accepted: 27 10 2020
entrez: 22 2 2021
pubmed: 23 2 2021
medline: 23 2 2021
Statut: epublish

Résumé

Most of the approximately 60 genes that if mutated cause steroid-resistant nephrotic syndrome (SRNS) are highly expressed in the glomerular podocyte, rendering SRNS a "podocytopathy." We performed whole-exome sequencing (WES) in 1200 nephrotic syndrome (NS) patients. We discovered homozygous truncating and homozygous missense mutation in We show that SYNPO2 variants may lead to Rac1-ARP3 dysregulation, and may play a role in the pathogenesis of nephrotic syndrome.

Identifiants

pubmed: 33615072
doi: 10.1016/j.ekir.2020.10.040
pii: S2468-0249(20)31711-3
pmc: PMC7879128
doi:

Types de publication

Journal Article

Langues

eng

Pagination

472-483

Subventions

Organisme : NCATS NIH HHS
ID : UL1 TR001863
Pays : United States
Organisme : NIDDK NIH HHS
ID : P30 DK079310
Pays : United States
Organisme : NIDDK NIH HHS
ID : R01 DK076683
Pays : United States
Organisme : NIDDK NIH HHS
ID : T32 DK007726
Pays : United States
Organisme : NIDDK NIH HHS
ID : F32 DK122766
Pays : United States

Informations de copyright

© 2020 International Society of Nephrology. Published by Elsevier Inc.

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Auteurs

Youying Mao (Y)

Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Department of Nephrology, Shanghai Children's Medical Center, Shanhai Jiaotong University, Shanghai, China.

Ronen Schneider (R)

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

Peter F M van der Ven (PFM)

Institute for Cell Biology, Department of Molecular Cell Biology, University of Bonn, Bonn, Germany.

Marvin Assent (M)

Institute for Cell Biology, Department of Molecular Cell Biology, University of Bonn, Bonn, Germany.

Keerthika Lohanadan (K)

Institute for Cell Biology, Department of Molecular Cell Biology, University of Bonn, Bonn, Germany.

Verena Klämbt (V)

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

Florian Buerger (F)

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

Thomas M Kitzler (TM)

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

Konstantin Deutsch (K)

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

Makiko Nakayama (M)

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

Amar J Majmundar (AJ)

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

Nina Mann (N)

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

Tobias Hermle (T)

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

Ana C Onuchic-Whitford (AC)

Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
Renal Division, Brigham and Women's Hospital, Harvard Medical School, Boston, Massachusetts, USA.

Wei Zhou (W)

Department of Nephrology, Shanghai Children's Medical Center, Shanhai Jiaotong University, Shanghai, China.

Nandini Nagarajan Margam (NN)

Department of Microbiology and Immunology, Dalhousie University, Halifax, Nova Scotia, Canada.

Roy Duncan (R)

Department of Microbiology and Immunology, Dalhousie University, Halifax, Nova Scotia, Canada.

Jonathan Marquez (J)

Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut, USA.

Mustafa Khokha (M)

Department of Pediatrics, Yale University School of Medicine, New Haven, Connecticut, USA.

Hanan M Fathy (HM)

Department of Pediatrics, Alexandria Faculty of medicine, Alexandria University, Alexandria, Egypt.

Jameela A Kari (JA)

Department of Pediatrics, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia.
Pediatric Nephrology Center of Excellence, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia.

Sherif El Desoky (S)

Department of Pediatrics, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia.
Pediatric Nephrology Center of Excellence, King Abdulaziz University, Jeddah, Kingdom of Saudi Arabia.

Loai A Eid (LA)

Pediatric Nephrology Department, Dubai Kidney Center of Excellence, Dubai Hospital, Dubai, United Arab Emirates.

Hazem Subhi Awad (HS)

Pediatric Nephrology Department, Dubai Kidney Center of Excellence, Dubai Hospital, Dubai, United Arab Emirates.

Muna Al-Saffar (M)

Department of Pediatrics, Boston Children's Hospital, Harvard Medical School, Boston, Massachusetts, USA.
United Arab Emirates University, Abu Dhabi, United Arab Emirates.

Shrikant Mane (S)

Department of Genetics, Yale University School of Medicine, New Haven, Connecticut, USA.

Richard P Lifton (RP)

Department of Genetics, Yale University School of Medicine, New Haven, Connecticut, USA.
Howard Hughes Medical Institute, Chevy Chase, Maryland, USA.

Dieter O Fürst (DO)

Institute for Cell Biology, Department of Molecular Cell Biology, University of Bonn, Bonn, Germany.

Shirlee Shril (S)

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

Friedhelm Hildebrandt (F)

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

Classifications MeSH