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TitleCiliopathy patient variants reveal organelle-specific functions for TUBB4B in axonemal microtubules.
Journal, issue, pagesScience, Vol. 384, Issue 6694, Page eadf5489, Year 2024
Publish dateApr 26, 2024
AuthorsDaniel O Dodd / Sabrina Mechaussier / Patricia L Yeyati / Fraser McPhie / Jacob R Anderson / Chen Jing Khoo / Amelia Shoemark / Deepesh K Gupta / Thomas Attard / Maimoona A Zariwala / Marie Legendre / Diana Bracht / Julia Wallmeier / Miao Gui / Mahmoud R Fassad / David A Parry / Peter A Tennant / Alison Meynert / Gabrielle Wheway / Lucas Fares-Taie / Holly A Black / Rana Mitri-Frangieh / Catherine Faucon / Josseline Kaplan / Mitali Patel / Lisa McKie / Roly Megaw / Christos Gatsogiannis / Mai A Mohamed / Stuart Aitken / Philippe Gautier / Finn R Reinholt / Robert A Hirst / Chris O'Callaghan / Ketil Heimdal / Mathieu Bottier / Estelle Escudier / Suzanne Crowley / Maria Descartes / Ethylin W Jabs / Priti Kenia / Jeanne Amiel / Giacomo Maria Bacci / Claudia Calogero / Viviana Palazzo / Lucia Tiberi / Ulrike Blümlein / Andrew Rogers / Jennifer A Wambach / Daniel J Wegner / Anne B Fulton / Margaret Kenna / Margaret Rosenfeld / Ingrid A Holm / Alan Quigley / Emma A Hall / Laura C Murphy / Diane M Cassidy / Alex von Kriegsheim / / / / Jean-François Papon / Laurent Pasquier / Marlène S Murris / James D Chalmers / Claire Hogg / Kenneth A Macleod / Don S Urquhart / Stefan Unger / Timothy J Aitman / Serge Amselem / Margaret W Leigh / Michael R Knowles / Heymut Omran / Hannah M Mitchison / Alan Brown / Joseph A Marsh / Julie P I Welburn / Shih-Chieh Ti / Amjad Horani / Jean-Michel Rozet / Isabelle Perrault / Pleasantine Mill /
PubMed AbstractTubulin, one of the most abundant cytoskeletal building blocks, has numerous isotypes in metazoans encoded by different conserved genes. Whether these distinct isotypes form cell type- and context- ...Tubulin, one of the most abundant cytoskeletal building blocks, has numerous isotypes in metazoans encoded by different conserved genes. Whether these distinct isotypes form cell type- and context-specific microtubule structures is poorly understood. Based on a cohort of 12 patients with primary ciliary dyskinesia as well as mouse mutants, we identified and characterized variants in the isotype that specifically perturbed centriole and cilium biogenesis. Distinct variants differentially affected microtubule dynamics and cilia formation in a dominant-negative manner. Structure-function studies revealed that different TUBB4B variants disrupted distinct tubulin interfaces, thereby enabling stratification of patients into three classes of ciliopathic diseases. These findings show that specific tubulin isotypes have distinct and nonredundant subcellular functions and establish a link between tubulinopathies and ciliopathies.
External linksScience / PubMed:38662826 / PubMed Central
MethodsEM (single particle)
Resolution2.8 Å
Structure data

40480

8sh7

EMDB-40480, PDB-8sh7:
TUBB4B and TUBA1A Heterodimer from Human Respiratory Doublet Microtubules
Method: EM (single particle) / Resolution: 2.8 Å

Chemicals

ChemComp-GTP:
GUANOSINE-5'-TRIPHOSPHATE / GTP, energy-carrying molecule*YM

ChemComp-MG:
Unknown entry

ChemComp-GDP:
GUANOSINE-5'-DIPHOSPHATE / GDP, energy-carrying molecule*YM

Source
  • homo sapiens (human)
KeywordsSTRUCTURAL PROTEIN / Cilia / Axoneme / Microtubule

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