Johannes N. Spelbrink

I. Holt, A. Spinazzola, M. Janssen, J. N. Spelbrink - Physician's Guide to the Diagnosis, Treatment, and Follow-Up of Inherited Metabolic Diseases, 2022

Cited by 0 Semantic Scholar

Top3α is the replicative topoisomerase in mitochondrial DNA replication OA

A. Hangas, Nina J Kekäläinen, Alisa Potter, Craig T. Michell, Kauko J Aho - Nucleic Acids Research, 2022

Cited by 19 Semantic Scholar

Mitochondrial RNA processing defect caused by a SUPV3L1 mutation in two siblings with a novel neurodegenerative syndrome OA

Selma L. van Esveld, R. Rodenburg, Fathiya Al-Murshedi, E. Al-Ajmi, Sana Al‐Zuhaibi - Journal of Inherited Metabolic Disease, 2022

Cited by 12 Semantic Scholar

RNA Crosslinking to Analyze the Mitochondrial RNA-Binding Proteome.

Selma L. van Esveld, J. N. Spelbrink - Methods in molecular biology, 2021

Cited by 3 Semantic Scholar

A Combined Mass Spectrometry and Data Integration Approach to Predict the Mitochondrial Poly(A) RNA Interacting Proteome OA

Selma L. van Esveld, Şirin Cansız-Arda, Fenna Hensen, R. van der Lee, M. Huynen - Front. Cell Dev. Biol., 2019

Cited by 5 Semantic Scholar

Dominant mutations in mtDNA maintenance gene SSBP1 cause optic atrophy and foveopathy OA

Camille Piro-Mégy, Emmanuelle Sarzi, Aleix Tarrés-Solé, M. Péquignot, Fenna Hensen - Journal of Clinical Investigation, 2019

Cited by 55 Semantic Scholar

Research Topics

Mitochondrial Function and Pathology (83) Metabolism and Genetic Disorders (47) ATP Synthase and ATPases Research (29) RNA and protein synthesis mechanisms (17) RNA modifications and cancer (15)

Affiliations

University of Helsinki

FI 2005 - 2005

Radboud University Nijmegen

NL 2024 - 2011

Tampere University of Applied Sciences

FI 2009 - 2000

Tampere University

FI 2015 - 2002

University of Milan

IT 2001 - 2001

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Johannes N. Spelbrink

Publication & Citation Trends

Publications

Human SIRT5 variants with reduced stability and activity do not cause neuropathology in mice OA

Let's make it clear: systematic exploration of mitochondrial DNA– and RNA–protein complexes by complexome profiling OA

SIRT5 variants from patients with mitochondrial disease are associated with reduced SIRT5 stability and activity, but not with neuropathology OA

Uncharacterized protein C17orf80 – a novel interactor of human mitochondrial nucleoids OA

Disorders of Replication, Transcription and Translation of Mitochondrial DNA