Jorg Heierhorst

Dynll1 is essential for development and promotes endochondral bone formation by regulating intraflagellar dynein function in primary cilia

Ashleigh King, Nicolas C Hoch, Narelle E McGregor, Natalie A Sims, Ian M Smyth, Jorg Heierhorst

Journal article  |  2019  |  HUMAN MOLECULAR GENETICS

Mutations in subunits of the cilia-specific cytoplasmic dynein-2 (CD2) complex cause short-rib thoracic dystrophy syndromes (SRTDs..

ATP-dependent helicase activity is dispensable for the physiological functions of Recql4

Wilson Castillo-Tandazo, Monique F Smeets, Vincent Murphy, Rui Liu, Charlotte Hodson, Joerg Heierhorst, Andrew J Deans, Carl R Walkley

Journal article  |  2019  |  PLOS GENETICS

Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive disorder characterized by skin rash (poikiloderma), skeletal dysplas..

The ASCIZ-DYNLL1 axis promotes 53BP1-dependent non-homologous end joining and PARP inhibitor sensitivity

Jordan R Becker, Raquel Cuella-Martin, Marco Barazas, Rui Liu, Catarina Oliveira, Antony W Oliver, Kirstin Bilham, Abbey B Holt, Andrew N Blackford, Jorg Heierhorst, Jos Jonkers, Sven Rottenberg, J Ross Chapman

Journal article  |  2018  |  NATURE COMMUNICATIONS

53BP1 controls a specialized non-homologous end joining (NHEJ) pathway that is essential for adaptive immunity, yet oncogenic in B..

Multivalency regulates activity in an intrinsically disordered transcription factor

Sarah Clark, Janette B Myers, Ashleigh King, Radovan Fiala, Jiri Novacek, Grant Pearce, Jorg Heierhorst, Steve L Reichow, Elisar J Barbar

Journal article  |  2018  |  ELIFE

The transcription factor ASCIZ (ATMIN, ZNF822) has an unusually high number of recognition motifs for the product of its main targ..

Proapoptotic BIM Impacts B Lymphoid Homeostasis by Limiting the Survival of Mature B Cells in a Cell-Autonomous Manner

Rui Liu, Ashleigh King, Philippe Bouillet, David M Tarlinton, Andreas Strasser, Jorg Heierhorst

Journal article  |  2018  |  FRONTIERS IN IMMUNOLOGY

The proapoptotic BH3-only protein BIM (Bcl2l11) plays key roles in the maintenance of multiple hematopoietic cell types. In mice, ..

Dynein light chain regulates adaptive and innate B cell development by distinctive genetic mechanisms

Ashleigh King, Lingli Li, David M Wong, Rui Liu, Rebecca Bamford, Andreas Strasser, David M Tarlinton, Jorg Heierhorst

Journal article  |  2017  |  PLOS GENETICS

Mechanistic differences in the development and function of adaptive, high-affinity antibody-producing B-2 cells and innate-like, "..

ASCIZ/ATMIN is dispensable for ATM signaling in response to replication stress

Rui Liu, Ashleigh King, Nicolas C Hoch, Catherine Chang, Gemma L Kelly, Andrew J Deans, Jorg Heierhorst

Journal article  |  2017  |  DNA REPAIR

The ATM kinase plays critical roles in the response to DNA double-strand breaks, and can also be activated by prolonged DNA replic..

Mechanism of Ubiquitination and Deubiquitination in the Fanconi Anemia Pathway

Sylvie van Twest, Vincent J Murphy, Charlotte Hodson, Winnie Tan, Paolo Swuec, Julienne J O'Rourke, Jorg Heierhorst, Wayne Crismani, Andrew J Deans

Journal article  |  2017  |  MOLECULAR CELL

Monoubiquitination and deubiquitination of FANCD2:FANCI heterodimer is central to DNA repair in a pathway that is defective in the..

Quantitative Analysis of Yeast Checkpoint Protein Kinase Activity by Combined Mass Spectrometry Enzyme Assays

NC Hoch, ES-W Chen, M-D Tsai, J Heierhorst

Book Chapter  |  2017  |  Methods in Enzymology

Virtually all eukaryotic cell functions and signaling pathways are regulated by protein phosphorylation. The Rad53 kinase plays cr..

The Transcription Factor ASCIZ and Its Target DYNLL1 Are Essential for the Development and Expansion of MYC-Driven B Cell Lymphoma

David M Wong, Lingli Li, Sabine Jurado, Ashleigh King, Rebecca Bamford, Meaghan Wall, Mannu K Walia, Gemma L Kelly, Carl R Walkley, David M Tarlinton, Andreas Strasser, Joerg Heierhorst

Journal article  |  2016  |  CELL REPORTS

How MYC promotes the development of cancer remains to be fully understood. Here, we report that the Zn(2+)-finger transcription fa..