M
Marris G. Dibley
Researcher at Discovery Institute
Publications - 6
Citations - 614
Marris G. Dibley is an academic researcher from Discovery Institute. The author has contributed to research in topics: Protein subunit & Electron Transport Complex I. The author has an hindex of 5, co-authored 5 publications receiving 408 citations. Previous affiliations of Marris G. Dibley include Monash University.
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Journal ArticleDOI
Accessory subunits are integral for assembly and function of human mitochondrial complex I
David A. Stroud,Elliot Surgenor,Luke E. Formosa,Luke E. Formosa,Boris Reljic,Ann E. Frazier,Marris G. Dibley,Laura D. Osellame,Tegan Stait,Traude H. Beilharz,David R. Thorburn,David R. Thorburn,Agus Salim,Michael T. Ryan +13 more
TL;DR: It is shown that 25 subunits are strictly required for assembly of a functional complex and 1 subunit is essential for cell viability, and coupling gene-editing technology with proteomics represents a powerful tool for dissecting large multi-subunit complexes and enables the study of complex dysfunction at a cellular level.
Journal ArticleDOI
Building a complex complex: Assembly of mitochondrial respiratory chain complex I.
TL;DR: This review outlines the current understanding of complex I biogenesis, with an emphasis on the assembly factors that facilitate the building of this architectural giant.
Journal ArticleDOI
The Mitochondrial Acyl-carrier Protein Interaction Network Highlights Important Roles for LYRM Family Members in Complex I and Mitoribosome Assembly
Marris G. Dibley,Luke E. Formosa,Baobei Lyu,Boris Reljic,Dylan McGann,Linden Muellner-Wong,Felix Kraus,Alice J. Sharpe,David A. Stroud,Michael T. Ryan +9 more
TL;DR: Subsequent knockout and interaction network studies revealed the LYRM member AltMiD51 to be important for optimal assembly of the large mitoribosome subunit, consistent with recent structural studies and emphasizes the common and essential role of NDUFAB1 as a protagonist in mitochondrial metabolism.
Journal ArticleDOI
Bi-allelic Mutations in NDUFA6 Establish Its Role in Early-Onset Isolated Mitochondrial Complex I Deficiency
Charlotte L. Alston,Juliana Heidler,Marris G. Dibley,Laura S. Kremer,Lucie S. Taylor,Carl Fratter,Courtney E. French,Ruth I. C. Glasgow,René G. Feichtinger,Isabelle Delon,Alistair T. Pagnamenta,Helen Dolling,Helen Dolling,Hugh Lemonde,Neil Aiton,Alf Bjornstad,Lisa Henneke,Jutta Gärtner,Holger Thiele,Katerina Tauchmannova,Gerardine Quaghebeur,Josef Houstek,Wolfgang Sperl,F. Lucy Raymond,F. Lucy Raymond,Holger Prokisch,Johannes A. Mayr,Robert McFarland,Joanna Poulton,Michael T. Ryan,Ilka Wittig,Marco Henneke,Robert W. Taylor +32 more
TL;DR: Interestingly, RNA-sequencing data provided evidence that the consensus RefSeq accession number does not correspond to the predominant transcript in clinically relevant tissues, prompting revision of the NDUFA6 RefSequ transcript and highlighting not only the importance of thorough variant interpretation but also the assessment of appropriate transcripts for analysis.
Journal ArticleDOI
A novel isoform of the human mitochondrial complex I subunit NDUFV3.
TL;DR: NDUFV3 represents the first known complex I subunit present in two functional isoforms, and is revealed to be present in both the canonical 10 kDa and a novel 50 kDa isoform, generated through alternative splicing.