The Pleiotropic Nuclear Envelope

A Biochemical Society Focused Meeting
In the past two decades, our understanding of the nuclear envelope (NE) has been radically transformed. From being viewed as a simple membrane barrier with a lamin intermediate filament lining, the NE is now known to incorporate a signaling and regulatory interface as complex and dynamic as that of the plasma membrane. On the outer face of the NE, connections to cytoskeletal filaments direct cell and nuclear migration and mechanosignal transduction, whilst connections to chromatin on its inner face direct genome regulation and stability. The importance of these various roles is highlighted by the association of NE protein mutations with human diseases ranging from muscular dystrophies to neuropathies, lipodystrophies and premature ageing syndromes.

Additionally, emerging technologies are finally enabling the tackling of longstanding questions such as the higher-order structure of the lamin polymer, nuclear membrane synthesis and dynamics, and membrane protein complexes. Many ER proteins are also now being found to contribute to NE dynamics and investigations of pathogen interactions with the NE are yielding yet further insights into this complex structure. This incredible diversity of functions and structure-function relationships underscores the pleiotropy of NE-linked diseases and in this programme we draw upon experts in biophysics, biochemistry, cell biology, microscopy, and virology to highlight the wide breadth of NE functions. By bringing together diverse groups covering a wide range of expertise, from fluorescence fluxuation microscopy to crosslinking mass spectrometry to human genetics, we expect to promote a dialogue that may better elucidate NE disease mechanisms.

Topics include:
▪ New directions in nuclear envelopathies
▪ Dynamic nucleo-cytoskeletal interactions and mechanotranduction
▪ Genome regulation from the nuclear envelope
▪ New nuclear envelope signaling pathways and mechanisms
▪ Detailed structure of the lamina and membrane complexes
▪ Nuclear membrane biosynthesis and dynamics
▪ Pathogen interactions with the nuclear membrane

Organizers:
Sue Shackleton (University of Leicester, United Kingdom)
Eric Schirmer (University of Edinburgh, United Kingdom)
Edgar Gomes (University of Lisbon , Portugal)
Tuesday 22 August 2017
16:30 Registration

17:00 ‘Welcome’ drinks reception

18:00 – 19:00
Susan Gasser (Friedrich Miescher Institute, Switzerland)

Session 1: New Directions in Nuclear Envelopathies

Wednesday 23 August 2017
09:00 – 09:30
Therapies for nuclear envelope-linked dystrophies
Gisele Bonne (UM76 – UPMC Univ. Paris 6 / U974 – Inserm / UMR7215 – CNRS, France)
09:30 – 10:00
Nesprins: roles in nuclear envelope organisation, myogensis and disease
Qiuping Zhang (King’s College London, United Kingdom)
10:00 – 10:15
Myotonic Dystrophy: links to the nuclear envelope
Selected Oral Communication – Peter Meinke (Friedrich-Baur-Institut, Germany)
10:15 – 10:30
Nuclear defects in patients with inherited diseases of the skeletal muscle contractile filaments
Selected Oral Communication – Jacob Ross (Kings College London, UK)
10:30 – 11:00 Refreshment Break

11:00 – 11:15
Deep coverage microscopy reveals aberrant nuclear dynamics in laminopathy model cells
Selected Oral Communication – Winnok De Vos (Antwerp University, Belgium)
11:15 – 11:30
POPDC3, a member of the Popeye domain containing gene family, is associated with a centronuclear myopathy-like phenotype
Selected Oral Communication – Thomas Brand (Imperial College London, UK)
11:30 – 11:55
CRISPR mouse models
Matt Wheeler (Cyagen)
11:55 – 12:20 Flash Poster Presentations

12:20 – 14:30 Lunch and Poster Session 1

Session 2: Dynamic Nucleo-cytoskeletal Interactions and mechanotransduction

Wednesday 23 August 2017
14:30 – 14:55
Squeezing the nucleus to the periphery of skeletal myofibers
Edgar Gomes (University of Lisbon, Portugal)
14:55 – 15:15
LINCing myonuclei to the microtubule network and its role in muscular dystrophy
Sue Shackleton (University of Leicester, UK)
15:15 – 15:30
Emery-Dreifuss Muscular Dystrophy-linked genes and Centronuclear myopathy-linked genes regulate myonuclear movement by distinct mechanisms
Selected Oral Communication – Eric Folker (Boston College, USA)
15:30 – 15:45
Nuclear decoupling and chaperones protect cell homeostasis during mechanical loading
Selected Oral Communication – Hamish Gilbert (University of Manchester, UK)
15:45 – 16:00
Multiple isoforms of nesprin1 are integral components of ciliary rootlets
Selected Oral Communication – Didier Hodzic (Washington University, USA)
16:00 – 16:30 Refreshment Break

Session 3: Genome Regulation from the Nuclear Envelope

Wednesday 23 August 2017
16:30 – 17:00
Gene positioning at the nuclear periphery
Wendy Bickmore (IGMM, United Kingdom)
17:00 – 17:30
A-type nuclear lamins and regulation of spatial genome conformation
Philippe Collas (University of Oslo, Norway)
17:30 – 17:45
Imaging chromatin dynamics reveals a novel mechanism for nuclear organisation after cell division
Selected Oral Communication – Alice Sherrard (University of Bristol, UK)
17:45 – 18:00
Epigenetic maintenance of genome architecture by nuclear pore proteins
Selected Oral Communication – Maya Capelson (University of Pennsylvania, Philadelphia, USA)
18:00 – 18:15
Concomitant nuclear localization of gene loci and functional distribution of their encoded proteins
Selected Oral Communication – Steven Kosak (Northwestern University, Chicago, USA)

Session 4: New Signalling Pathways and Mechanisms

Thursday 24 August 2017
09:00 – 09:30
ERK signalling in lamin-associated cardiomyopathy
Howard Worman (Columbia University, U.S.A.)
09:30 – 10:00
RepoMan regulation of nuclear membrane proteins
Paola Vagnarelli (Brunel University London, United Kingdom)
10:00 – 10:15
super resolution approaches
TBC (Zeiss)
10:15 – 10:45 Refreshment Break

10:45 – 11:00
Distinct nuclear transport pathways for inner nuclear membrane proteins revealed by super-resolution microscopy
Selected Oral Communication – Weidong Yang (Temple University, Philadelphia, USA)
11:00 – 11:30
Translocation through the peripheral NPC channels
Ulrike Kutay (ETH Zurich, Switzerland)
11:30 – 12:00 Flash Poster Presentations

12:00 – 14:15 Lunch and Poster Session 2

Session 5: Detailed Structure of the Lamina and Membrane Complexes

Thursday 24 August 2017
14:15 – 14:45
Herpesvirus nuclear egress – the prototypic vesicular nucleo-cytoplasmic transport
Thomas Mettenleiter (Friedrich-Loeffler-Institut, Germany)
14:45 – 15:00
Bead-induced nuclear envelope assembly in the living cell
Selected Oral Communication – Tokuko Haraguchi (Advanced ICT Research Institute Kobe, NICT, Japan)
15:00 – 15:30
EMBO Young investigator Lecture
Protein turnover at the nuclear envelope
Pedro Carvalho (Centre for Genomic Regulation, Spain)
15:30 – 16:00 Refreshment Break

Session 6: Progeria Sponsored by the Society of Biologists

Thursday 24 August 2017
16:00 – 16:30
Signaling changes in Progeria
Maria Eriksson (Karolinska Institutet , Sweden)
16:30 – 16:45
Are molecular defects identified by a prelamin A mediated cardiomyopathy model recapitulated in human cardiomyopathy?
Selected Oral Communication – Daniel Brayson (King’s College London, London, UK)
16:45 – 17:00
Transcription factor Prep1 is a novel regulator of nuclear structure and stablity
Selected Oral Communication – Divya Purushothaman (IFOM, Milano, Italy)
17:00 – 17:30
Keynote Progeria: A paradigm for modern biomedical research
Tom Misteli (National Cancer Institute, U.S.A.)
19:00 Conference Dinner: John McIntyre Conference Centre

Session 7: Nuclear Membrane Biosynthesis and Dynamics

Friday 25 August 2017
09:00 – 09:30
FFS quantification of NET complexes
GW Gant Luxton (University of Minnesota, U.S.A.)
09:30 – 10:00
CLMS to determine lamin structure
Eric Schrimer (Edinburgh University, UK)
10:00 – 10:15
Using C. elegans to dissect lamin-mediated nuclear envelope repair mechanisms in vivo
Selected Oral Communication – Shirin Bahmanyar (Yale University, USA)
10:15 – 10:30
A three-dimensional view of the interface between nuclear envelope and chromatin
Selected Oral Communication – Camille Samson (CEA Saclay, Gif-Sur-Yvette, France)
10:30 – 11:00 Refreshment Break

Session 8: Potpourri of Nuclear Envelope Functions

Friday 25 August 2017
11:00 – 11:30
NanoSIMS shows de novo lipid synthesis in the nucleus
David Vaux (Oxford University, United Kingdom)
11:30 – 11:45
Torsins on the inner nuclear membrane provide evolutionarily conserved control over cellular lipid metabolism
Selected Oral Communication – Rose Goodchild (VIB, Belgium)
11:45 – 12:00
ESCRTs at the nuclear envelope: for better or worse
Selected Oral Communication – Coen Campsteijn (University of Oslo, Oslo, Norway)
12:00 – 12:30
ESCRTs in nuclear envelope dynamics
Jeremy Carlton (King’s College London, United Kingdom)
12:30 Meeting Close

Date

Aug 22 2017 - Aug 25 2017

Time

All of the day