Homozygous EPRS1 missense variant causing hypomyelinating leukodystrophy-15 alters variant-distal mRNA m


Journal

Nature communications
ISSN: 2041-1723
Titre abrégé: Nat Commun
Pays: England
ID NLM: 101528555

Informations de publication

Date de publication:
20 May 2024
Historique:
received: 15 10 2023
accepted: 03 05 2024
medline: 21 5 2024
pubmed: 21 5 2024
entrez: 20 5 2024
Statut: epublish

Résumé

Hypomyelinating leukodystrophy (HLD) is an autosomal recessive disorder characterized by defective central nervous system myelination. Exome sequencing of two siblings with severe cognitive and motor impairment and progressive hypomyelination characteristic of HLD revealed homozygosity for a missense single-nucleotide variant (SNV) in EPRS1 (c.4444 C > A; p.Pro1482Thr), encoding glutamyl-prolyl-tRNA synthetase, consistent with HLD15. Patient lymphoblastoid cell lines express markedly reduced EPRS1 protein due to dual defects in nuclear export and cytoplasmic translation of variant EPRS1 mRNA. Variant mRNA exhibits reduced METTL3 methyltransferase-mediated writing of N

Identifiants

pubmed: 38769304
doi: 10.1038/s41467-024-48549-x
pii: 10.1038/s41467-024-48549-x
doi:

Substances chimiques

N-methyladenosine 0
METTL3 protein, human 0
YTHDC1 protein, human 0
YTHDF1 protein, human 0

Types de publication

Journal Article

Langues

eng

Sous-ensembles de citation

IM

Pagination

4284

Subventions

Organisme : NIDDK NIH HHS
ID : R01 DK124203
Pays : United States
Organisme : NIDDK NIH HHS
ID : R01 DK130377
Pays : United States
Organisme : NIA NIH HHS
ID : R01 AG067146
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS124547
Pays : United States
Organisme : NINDS NIH HHS
ID : R01 NS124581
Pays : United States

Informations de copyright

© 2024. The Author(s).

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Auteurs

Debjit Khan (D)

Department of Cardiovascular and Metabolic Sciences, Cleveland Clinic, Lerner Research Institute, Cleveland, OH, USA.

Iyappan Ramachandiran (I)

Department of Cardiovascular and Metabolic Sciences, Cleveland Clinic, Lerner Research Institute, Cleveland, OH, USA.

Kommireddy Vasu (K)

Department of Cardiovascular and Metabolic Sciences, Cleveland Clinic, Lerner Research Institute, Cleveland, OH, USA.

Arnab China (A)

Department of Cardiovascular and Metabolic Sciences, Cleveland Clinic, Lerner Research Institute, Cleveland, OH, USA.

Krishnendu Khan (K)

Department of Cardiovascular and Metabolic Sciences, Cleveland Clinic, Lerner Research Institute, Cleveland, OH, USA.

Fabio Cumbo (F)

Genomic Medicine Institute, Cleveland Clinic, Lerner Research Institute, Cleveland, OH, USA.

Dalia Halawani (D)

Department of Cardiovascular and Metabolic Sciences, Cleveland Clinic, Lerner Research Institute, Cleveland, OH, USA.

Fulvia Terenzi (F)

Department of Cardiovascular and Metabolic Sciences, Cleveland Clinic, Lerner Research Institute, Cleveland, OH, USA.

Isaac Zin (I)

Department of Cardiovascular and Metabolic Sciences, Cleveland Clinic, Lerner Research Institute, Cleveland, OH, USA.
Department of Chemistry, Cleveland State University, Cleveland, OH, USA.

Briana Long (B)

Department of Cardiovascular and Metabolic Sciences, Cleveland Clinic, Lerner Research Institute, Cleveland, OH, USA.

Gregory Costain (G)

Department of Paediatrics, Division of Clinical and Metabolic Genetics, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.

Susan Blaser (S)

Department of Diagnostic Imaging, Division of Neuroradiology, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.

Amanda Carnevale (A)

Department of Paediatrics, Division of Clinical and Metabolic Genetics, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada.

Valentin Gogonea (V)

Department of Chemistry, Cleveland State University, Cleveland, OH, USA.

Ranjan Dutta (R)

Department of Neuroscience, Cleveland Clinic, Lerner Research Institute, Cleveland, OH, USA.

Daniel Blankenberg (D)

Genomic Medicine Institute, Cleveland Clinic, Lerner Research Institute, Cleveland, OH, USA.

Grace Yoon (G)

Department of Paediatrics, Division of Clinical and Metabolic Genetics, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada. grace.yoon@utoronto.ca.
Department of Paediatrics, Division of Neurology, The Hospital for Sick Children, University of Toronto, Toronto, ON, Canada. grace.yoon@utoronto.ca.

Paul L Fox (PL)

Department of Cardiovascular and Metabolic Sciences, Cleveland Clinic, Lerner Research Institute, Cleveland, OH, USA. foxp@ccf.org.

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