Supernumerary proteins of the human mitochondrial ribosomal small subunit are integral for assembly and translation.

biochemistry biological sciences cell biology molecular biology

Journal

iScience
ISSN: 2589-0042
Titre abrégé: iScience
Pays: United States
ID NLM: 101724038

Informations de publication

Date de publication:
19 Jul 2024
Historique:
received: 23 06 2022
revised: 28 03 2024
accepted: 01 06 2024
medline: 17 7 2024
pubmed: 17 7 2024
entrez: 17 7 2024
Statut: epublish

Résumé

Mitochondrial ribosomes (mitoribosomes) have undergone substantial evolutionary structural remodeling accompanied by loss of ribosomal RNA, while acquiring unique protein subunits located on the periphery. We generated CRISPR-mediated knockouts of all 14 unique (mitochondria-specific/supernumerary) human mitoribosomal proteins (snMRPs) in the small subunit to study the effect on mitoribosome assembly and protein synthesis, each leading to a unique mitoribosome assembly defect with variable impact on mitochondrial protein synthesis. Surprisingly, the stability of mS37 was reduced in all our snMRP knockouts of the small and large ribosomal subunits and patient-derived lines with mitoribosome assembly defects. A redox-regulated CX

Identifiants

DOI: 10.1016/j.isci.2024.110185 PMID: 39015150 PMC: PMC11251090
pubmed: 39015150
doi: 10.1016/j.isci.2024.110185
pii: S2589-0042(24)01410-X
pmc: PMC11251090
doi:

Types de publication

Journal Article

Langues

eng

Pagination

110185

Informations de copyright

© 2024 The Authors.

Déclaration de conflit d'intérêts

The authors declare no competing interests.

Auteurs

Taru Hilander (T)

Genetics Section, Cardiovascular and Genomics Research Institute, St George's, University of London, London, UK.

Ryan Awadhpersad (R)

Department of Biochemistry and Developmental Biology, Faculty of Medicine, University of Helsinki, Helsinki, Finland.

Geoffray Monteuuis (G)

Department of Biochemistry and Developmental Biology, Faculty of Medicine, University of Helsinki, Helsinki, Finland.

Krystyna L Broda (KL)

Genetics Section, Cardiovascular and Genomics Research Institute, St George's, University of London, London, UK.

Max Pohjanpelto (M)

Genetics Section, Cardiovascular and Genomics Research Institute, St George's, University of London, London, UK.
Stem Cells and Metabolism Research Program, Faculty of Medicine, University of Helsinki, 00290 Helsinki, Finland.

Elizabeth Pyman (E)

Genetics Section, Cardiovascular and Genomics Research Institute, St George's, University of London, London, UK.

Sachin Kumar Singh (SK)

Department of Immunology, Institute of Clinical Medicine, University of Oslo and Oslo University Hospital, Oslo, Norway.

Tuula A Nyman (TA)

Department of Immunology, Institute of Clinical Medicine, University of Oslo and Oslo University Hospital, Oslo, Norway.

Isabelle Crevel (I)

Core Facilities, St George's, University of London, London, UK.

Robert W Taylor (RW)

Mitochondrial Research Group, Translational and Clinical Research Institute, Faculty of Medical Sciences, Newcastle University, Newcastle upon Tyne NE2 4HH, UK.
NHS Highly Specialised Service for Rare Mitochondrial Disorders, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne NE1 4LP, UK.

Ann Saada (A)

Department of Genetics, Hadassah Medical Center & Faculty of Medicine, Hebrew University of Jerusalem, Jerusalem 9112001 Israel.

Diego Balboa (D)

Stem Cells and Metabolism Research Program, Faculty of Medicine, University of Helsinki, 00290 Helsinki, Finland.

Brendan J Battersby (BJ)

Institute of Biotechnology, HiLIFE, University of Helsinki, Helsinki, Finland.

Christopher B Jackson (CB)

Department of Biochemistry and Developmental Biology, Faculty of Medicine, University of Helsinki, Helsinki, Finland.

Christopher J Carroll (CJ)

Genetics Section, Cardiovascular and Genomics Research Institute, St George's, University of London, London, UK.

Classifications MeSH