Web Desc
Conformational Diseases of the Secretory Pathway
Organizer(s): Randal J. Kaufman and Tom A. Rapoport
Date: March 01 - 06, 2003
Location: Sagebrush Inn & Suites, Taos, NM, USA
Sponsored in part by The Director's Sponsor Fund
For important information on the coronavirus, please click here
Summary of Meeting:
In eukaryotes, nascent unfolded polypeptides achieve conformational maturation in the endoplasmic reticulum prior to cell surface expression or secretion. Particular mutations in the primary amino acid sequence can hinder the attainment of native protein structure, causing protein folding and quality control systems in the early secretory pathway to participate in the molecular pathogenesis of those human diseases where there is selective degradation or accumulation of a structurally-alerted gene product. The conference will focus on recent experimental findings that have begun to more fully dissect the complexity and diversity of these systems. The objectives are to stimulate a multi-disciplinary understanding of how specific covalent modifications and molecular chaperones facilitate protein folding, elucidate the repertoire of conformation-based monitoring and disposal systems, and define the complexity of cellular responses to the accumulation of aberrant molecules. An outcome will be the design of pharmacological strategies to enhance the intracellular transport of potentially salvageable molecules.
Discounted Abstract Deadline: November 1 2002
Discounted Registration Deadline: January 3 2003
We gratefully acknowledge additional support for this conference from:

Affinity BioReagents, Inc.
We gratefully acknowledge the generous grant for this conference provided by:

National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK)
Grant No. 1 R13 DK063935-01
We appreciate the organizations that provide Keystone Symposia with additional support, such as marketing and advertising:

Click here to view more of these organizations
Special thanks to the following for their support of Keystone Symposia initiatives to increase participation at this meeting by scientists from underrepresented backgrounds:

Click here to view more of these organizations

Program

Saturday, March 01 | 3:00PM - 7:00PM
Registration
Room: Chamisa Lobby


Saturday, March 01 | 6:15PM - 7:15PM
Welcome
Room: Chamisa Lobby


Saturday, March 01 | 7:15PM - 7:30PM
Orientation
Room: Chamisa 1


Saturday, March 01 | 7:30PM - 8:30PM
Keynote Addresses
Room: Chamisa 1

Speaker 1 of 1
Joseph L. Goldstein, University of Texas Southwestern Medical Center, USA
Central Role of ER-to-Golgi Transport in the Regulation of Cholesterol Homeostasis

Sunday, March 02 | 7:00AM - 8:00AM
Breakfast
Room: Los Vaqueros


Sunday, March 02 | 8:00AM - 11:00AM
The Endoplasmic Reticulum
Room: Chamisa 1

Speaker 1 of 4
Tom A. Rapoport, Harvard University Medical School, USA
Retro-Translocation of Proteins Out of the ER

Sunday, March 02 | 8:00AM - 11:00AM
The Endoplasmic Reticulum
Room: Chamisa 1

Speaker 2 of 4
* Randy W. Schekman, University of California at Berkeley, USA
Molecular Mechanism of Cargo Protein Capture by the COPII Coat

Sunday, March 02 | 8:00AM - 11:00AM
The Endoplasmic Reticulum
Room: Chamisa 1

Speaker 3 of 4
Ari Helenius, ETH Zurich, Switzerland
Folding and Quality Control in the ER

Sunday, March 02 | 8:00AM - 11:00AM
The Endoplasmic Reticulum
Room: Chamisa 1

Speaker 4 of 4
Ramanujan S. Hegde, MRC Laboratory of Molecular Biology, UK
Folding and Misfolding of the Prion Protein at the ER

Sunday, March 02 | 9:20AM - 9:40AM
Coffee Break
Room: Chamisa Lobby


Sunday, March 02 | 11:00AM - 1:00PM
Poster Setup
Room: Chamisa 2


Sunday, March 02 | 1:00PM - 10:00PM
Poster Viewing
Room: Chamisa 2


Sunday, March 02 | 4:30PM - 5:00PM
Coffee & Snacks Available
Room: Chamisa Lobby


Sunday, March 02 | 5:00PM - 7:00PM
The Contribution of N-Linked Glycans to Quality Control
Room: Chamisa 1

Speaker 1 of 4
* Karin Romisch, Saarland University, Germany
Coordination of Protein Translocation and N-Glycosylation at the ER Membrane

Sunday, March 02 | 5:00PM - 7:00PM
The Contribution of N-Linked Glycans to Quality Control
Room: Chamisa 1

Speaker 2 of 4
Markus Aebi, Swiss Federal Institute of Technology, Switzerland
N-Linked Protein Glycosylation in the ER: From Yeast to Congenital Disorders of Glycosylation

Sunday, March 02 | 5:00PM - 7:00PM
The Contribution of N-Linked Glycans to Quality Control
Room: Chamisa 1

Speaker 3 of 4
David B. Williams, University of Toronto, Canada
Calnexin and Calreticulin: Multifunctional Lectin-Chaperones of the Endoplasmic Reticulum

Sunday, March 02 | 5:00PM - 7:00PM
The Contribution of N-Linked Glycans to Quality Control
Room: Chamisa 1

Speaker 4 of 4
Richard N. Sifers, Baylor College of Medicine, USA
Short Talk: The Stochastic and Temporal Formation of a Glycoprotein
ERAD Signal Coordinates the Accuracy of Substrate Discrimination


Sunday, March 02 | 7:00PM - 8:00PM
Social Hour
Room: Chamisa 2


Sunday, March 02 | 8:00PM - 10:00PM
Poster Session 1
Room: Chamisa 2


Monday, March 03 | 7:00AM - 8:00AM
Breakfast
Room: Los Vaqueros


Monday, March 03 | 8:00AM - 11:00AM
Disulfide Bridge Formation in the ER
Room: Chamisa 1

Speaker 1 of 5
* Jonathan S. Weissman, University of California, San Francisco, USA
Mechanism of Oxidative Protein Folding in the Endoplasmic Reticulum

Monday, March 03 | 8:00AM - 11:00AM
Disulfide Bridge Formation in the ER
Room: Chamisa 1

Speaker 2 of 5
Chris Kaiser, Massachusetts Institute of Technology, USA
Pathways for Protein Disulfide Bond Formation

Monday, March 03 | 8:00AM - 11:00AM
Disulfide Bridge Formation in the ER
Room: Chamisa 1

Speaker 3 of 5
Ineke Braakman, Utrecht University, Netherlands
Folding and Disulfide Bond Formation in vitro and in vivo

Monday, March 03 | 8:00AM - 11:00AM
Disulfide Bridge Formation in the ER
Room: Chamisa 1

Speaker 4 of 5
Roberto M. Sitia, Universita' Vita-Salute San Raffaele, Italy
Short Talk: ERp44 is Involved in the ER Retention of Ero1-Lalpha and Cargo Proteins with Exposed Thiols

Monday, March 03 | 8:00AM - 11:00AM
Disulfide Bridge Formation in the ER
Room: Chamisa 1

Speaker 5 of 5
David M. Ferrari, Max Planck Society, Germany
Short Talk: Crystal Structures of the Protein Disulfide Isomerase-Related Proteins, Wind and ERp28

Monday, March 03 | 9:20AM - 9:40AM
Coffee Break
Room: Chamisa Lobby


Monday, March 03 | 11:00AM - 1:00PM
Poster Setup
Room: Chamisa 2


Monday, March 03 | 1:00PM - 10:00PM
Poster Viewing
Room: Chamisa 2


Monday, March 03 | 2:30PM - 4:30PM
Workshop 1: Protein Folding and Quality Control in the ER
Room: Chamisa 1

Speaker 1 of 10
* Richard N. Sifers, Baylor College of Medicine, USA

Monday, March 03 | 2:30PM - 4:30PM
Workshop 1: Protein Folding and Quality Control in the ER
Room: Chamisa 1

Speaker 2 of 10
* Linda M. Hendershot, St. Jude Children's Research Hospital, USA

Monday, March 03 | 2:30PM - 4:30PM
Workshop 1: Protein Folding and Quality Control in the ER
Room: Chamisa 1

Speaker 3 of 10
Robert D. Daniels, USA
N-Linked Glycans Direct the Co-Translational Folding Pathway of Influenza Hemagglutinin

Monday, March 03 | 2:30PM - 4:30PM
Workshop 1: Protein Folding and Quality Control in the ER
Room: Chamisa 1

Speaker 4 of 10
Gerardo Z. Lederkremer, Tel Aviv University, Israel
Separate Roles for Calnexin and ERp57 in ER Quality Control Revealed by Interactions with Asialoglycoprotein Receptor H2a and H2b

Monday, March 03 | 2:30PM - 4:30PM
Workshop 1: Protein Folding and Quality Control in the ER
Room: Chamisa 1

Speaker 5 of 10
Masato Takeuchi, Nara Institute of Science and Technology, Japan
Rot1p is a Hip-Like BiP/Kar2p Cofactor in the Yeast ER

Monday, March 03 | 2:30PM - 4:30PM
Workshop 1: Protein Folding and Quality Control in the ER
Room: Chamisa 1

Speaker 6 of 10
Bin Zhang, Cleveland Clinic Foundation, USA
Disruption of a Cargo-Specific ER to Golgi Transport Receptor Complex causes a Bleeding Disorder

Monday, March 03 | 2:30PM - 4:30PM
Workshop 1: Protein Folding and Quality Control in the ER
Room: Chamisa 1

Speaker 7 of 10
Douglas M. Cyr, University of North Carolina, Chapel Hill, USA
The Hsc70/CHIP Complex Functions as an E3 Ubiquitin Ligase to
Partition Nascent CFTR between Folding and Degradation Pathways


Monday, March 03 | 2:30PM - 4:30PM
Workshop 1: Protein Folding and Quality Control in the ER
Room: Chamisa 1

Speaker 8 of 10
Maurizio Molinari, Institute for Research in Biomedicine, Switzerland
A Role for EDEM in the Quality Control of Glycoproteins in the Mammalian ER

Monday, March 03 | 2:30PM - 4:30PM
Workshop 1: Protein Folding and Quality Control in the ER
Room: Chamisa 1

Speaker 9 of 10
Claude A. Jakob, ETH, Zurich Swiss Federal Institute of Technology, Switzerland
Ste24 Protein – a Zn2+-Dependent Metalloprotease at the ER Membrane – is Involved in ER-Associated Protein Degradation

Monday, March 03 | 2:30PM - 4:30PM
Workshop 1: Protein Folding and Quality Control in the ER
Room: Chamisa 1

Speaker 10 of 10
Nico P. Dantuma, Karolinska Institute, Sweden
Mutant Ubiquitin Found in Conformational Diseases Blocks Proteasomal Degradation

Monday, March 03 | 4:30PM - 5:00PM
Coffee & Snacks Available
Room: Chamisa Lobby


Monday, March 03 | 5:00PM - 7:00PM
Selective Folding and Export Processes
Room: Chamisa 1

Speaker 1 of 4
Davis Ng, National University of Singapore, Singapore
Distinct Pathways Define ER Quality Control Checkpoints

Monday, March 03 | 5:00PM - 7:00PM
Selective Folding and Export Processes
Room: Chamisa 1

Speaker 2 of 4
Charles K. Barlowe, Dartmouth Medical School, USA
Mechanisms of Protein Sorting in the Early Secretory Pathway

Monday, March 03 | 5:00PM - 7:00PM
Selective Folding and Export Processes
Room: Chamisa 1

Speaker 3 of 4
Ryosuke Takahashi, RIKEN Brain Science Institute, Japan
Familial Parkinson's Disease and Unfolded Protein Stress

Monday, March 03 | 5:00PM - 7:00PM
Selective Folding and Export Processes
Room: Chamisa 1

Speaker 4 of 4
Linda M. Hendershot, St. Jude Children's Research Hospital, USA
Short Talk: Regulating the Release of BiP from Unfolded Proteins

Monday, March 03 | 7:00PM - 8:00PM
Social Hour
Room: Chamisa 2


Monday, March 03 | 8:00PM - 10:00PM
Poster Session 2
Room: Chamisa 2


Tuesday, March 04 | 7:00AM - 8:00AM
Breakfast
Room: Los Vaqueros


Tuesday, March 04 | 8:00AM - 11:00AM
Proteolysis: ER-Associated Degradation and Death
Room: Chamisa 1

Speaker 1 of 5
Thomas Sommer, Max Delbrück Center for Molecular Medicine, Germany
Mechanisms and Principles of ER Associated Protein Degradation

Tuesday, March 04 | 8:00AM - 11:00AM
Proteolysis: ER-Associated Degradation and Death
Room: Chamisa 1

Speaker 2 of 5
Randy Y. Hampton, University of California, San Diego, USA
ER Ubiquitination and Cholesterol Regulation

Tuesday, March 04 | 8:00AM - 11:00AM
Proteolysis: ER-Associated Degradation and Death
Room: Chamisa 1

Speaker 3 of 5
* Ron R. Kopito, Stanford University, USA
Rhodopsin Misfolding and Aggregation in the Pathogenesis of Retinitis Pigmentosa

Tuesday, March 04 | 8:00AM - 11:00AM
Proteolysis: ER-Associated Degradation and Death
Room: Chamisa 1

Speaker 4 of 5
Per Noergaard, University of California, Berkeley, USA
Short Talk: Isolation of Yeast Sec61p Mutants Defective in Dislocation of Misfolded Proteins

Tuesday, March 04 | 8:00AM - 11:00AM
Proteolysis: ER-Associated Degradation and Death
Room: Chamisa 1

Speaker 5 of 5
Shoshana Bar-Nun, Tel Aviv University, Israel
Short Talk: Differential Roles of ER-Bound p97/Cdc48p and Proteasomes in Dislocations of Luminal ERAD Substrates

Tuesday, March 04 | 9:20AM - 9:40AM
Coffee Break
Room: Chamisa Lobby


Tuesday, March 04 | 11:00AM - 1:00PM
Poster Setup
Room: Chamisa 2


Tuesday, March 04 | 1:00PM - 10:00PM
Poster Viewing
Room: Chamisa 2


Tuesday, March 04 | 4:30PM - 5:00PM
Coffee & Snacks Available
Room: Chamisa Lobby


Tuesday, March 04 | 5:00PM - 7:00PM
Extraction/Release of Polypeptides from Membranes
Room: Chamisa 1

Speaker 1 of 3
Thomas Langer, CECAD Research Center, Germany
Quality Control of Membrane Proteins in Mitochondria

Tuesday, March 04 | 5:00PM - 7:00PM
Extraction/Release of Polypeptides from Membranes
Room: Chamisa 1

Speaker 2 of 3
Koreaki Ito, Kyoto University, Japan
Membrane-Bound Proteases of E.coli: Roles in Quality Control and Stress Responses

Tuesday, March 04 | 5:00PM - 7:00PM
Extraction/Release of Polypeptides from Membranes
Room: Chamisa 1

Speaker 3 of 3
Byron S. DeLaBarre, Agios Pharmaceuticals, USA
Short Talk: The X-ray Structure of Full Length VCP/p97

Tuesday, March 04 | 7:00PM - 8:00PM
Social Hour
Room: Chamisa 2


Tuesday, March 04 | 8:00PM - 10:00PM
Poster Session 3
Room: Chamisa 2


Wednesday, March 05 | 7:00AM - 8:00AM
Breakfast
Room: Los Vaqueros


Wednesday, March 05 | 8:00AM - 11:00AM
Cellular Response to Misfolded Protein
Room: Chamisa 1

Speaker 1 of 4
* David Ron, University of Cambridge, UK
The Integrated Stress Response: A Component of the Unfolded Protein Response

Wednesday, March 05 | 8:00AM - 11:00AM
Cellular Response to Misfolded Protein
Room: Chamisa 1

Speaker 2 of 4
Randal J. Kaufman, Sanford-Burnham Medical Research Institute, USA
The Physiological Role of the Unfolded Protein Response

Wednesday, March 05 | 8:00AM - 11:00AM
Cellular Response to Misfolded Protein
Room: Chamisa 1

Speaker 3 of 4
Kazutoshi Mori, Kyoto University, Japan
Roles of Transcriptional Induction Systems in the ER Quality Control

Wednesday, March 05 | 8:00AM - 11:00AM
Cellular Response to Misfolded Protein
Room: Chamisa 1

Speaker 4 of 4
Michael Karin, University of California, San Diego, USA
The Regulation of IKK and JNK Activity by Stress

Wednesday, March 05 | 9:20AM - 9:40AM
Coffee Break
Room: Chamisa Lobby


Wednesday, March 05 | 2:30PM - 4:30PM
Workshop 2: ER Stress in Normal Development and Disease
Room: Chamisa 1

Speaker 1 of 10
* Linda M. Hendershot, St. Jude Children's Research Hospital, USA

Wednesday, March 05 | 2:30PM - 4:30PM
Workshop 2: ER Stress in Normal Development and Disease
Room: Chamisa 1

Speaker 2 of 10
* Richard N. Sifers, Baylor College of Medicine, USA

Wednesday, March 05 | 2:30PM - 4:30PM
Workshop 2: ER Stress in Normal Development and Disease
Room: Chamisa 1

Speaker 3 of 10
Jingshi Shen, University of Colorado, Boulder, USA
Selective Dissociation of BiP from ATF6 Induced by ER Stress

Wednesday, March 05 | 2:30PM - 4:30PM
Workshop 2: ER Stress in Normal Development and Disease
Room: Chamisa 1

Speaker 4 of 10
Donalyn Scheuner, University of Michigan, USA
Dual Glucose Control of Translation in Pancreatic Beta Cells via (de)Phosphorylation of the Eukaryotic Initiation Factor eIF2alpha

Wednesday, March 05 | 2:30PM - 4:30PM
Workshop 2: ER Stress in Normal Development and Disease
Room: Chamisa 1

Speaker 5 of 10
Yanjun Ma, St. Jude Children's Research Hospital, USA
The Role of ATF4 in the Regulation of CHOP Transcription during Amino Acid Deprivation and ER Stress

Wednesday, March 05 | 2:30PM - 4:30PM
Workshop 2: ER Stress in Normal Development and Disease
Room: Chamisa 1

Speaker 6 of 10
Hideki Nishitoh, University of Tokyo, Japan
Molecular Mechanism of ER Stress-Induced JNK Activation and Apoptosis Through ASK1


Wednesday, March 05 | 2:30PM - 4:30PM
Workshop 2: ER Stress in Normal Development and Disease
Room: Chamisa 1

Speaker 7 of 10
Kyle W. Cunningham, Johns Hopkins University, USA
ER Stress Activates Calcium Signaling and Cell Death Pathways in Yeast

Wednesday, March 05 | 2:30PM - 4:30PM
Workshop 2: ER Stress in Normal Development and Disease
Room: Chamisa 1

Speaker 8 of 10
Bo Feng, Columbia University, USA
Cholesterol Accumulation In Macrophages Induces the Unfolded Protein Response

Wednesday, March 05 | 2:30PM - 4:30PM
Workshop 2: ER Stress in Normal Development and Disease
Room: Chamisa 1

Speaker 9 of 10
Alison E. Hall, University of Pennsylvania, USA
Potential Role for the Unfolded Protein Response in B Lymphocyte Negative Selection

Wednesday, March 05 | 2:30PM - 4:30PM
Workshop 2: ER Stress in Normal Development and Disease
Room: Chamisa 1

Speaker 10 of 10
Daniel J. Felmlee, University of Colorado Health Sciences Center, USA
Hepatitis C Virus induces ER stress; XBP-1 Converges Disease and Replication

Wednesday, March 05 | 4:30PM - 5:00PM
Coffee & Snacks Available
Room: Chamisa Lobby


Wednesday, March 05 | 5:00PM - 7:00PM
Aberrant Protein Folding in Human Disease
Room: Chamisa 1

Speaker 1 of 3
* Peter Arvan, Albert Einstein College of Medicine, USA
Thyroid Secretory Protein Misfolding and Degradation

Wednesday, March 05 | 5:00PM - 7:00PM
Aberrant Protein Folding in Human Disease
Room: Chamisa 1

Speaker 2 of 3
Richard C. Austin, McMaster University, Canada
Hyperhomocysteinemia, ER Stress and the Development of Atherosclerosis and Hepatic Steatosis

Wednesday, March 05 | 5:00PM - 7:00PM
Aberrant Protein Folding in Human Disease
Room: Chamisa 1

Speaker 3 of 3
Jennifer A. Lippincott-Schwartz, Howard Hughes Medical Institute - Janelia Farm, USA
Monitoring ER Associated Degradation (ERAD) in Single Live Cells using a Photoactivatable-GFP

Wednesday, March 05 | 7:00PM - 8:00PM
Social Hour
Room: Chamisa 2


Wednesday, March 05 | 8:00PM - 11:00PM
Entertainment
Room: Chamisa 2


Wednesday, March 05 | 8:00PM - 11:00PM
Cash Bar
Room: Chamisa 2


Thursday, March 06 | 10:21AM - 10:21AM
Departure


*Session Chair
†Invited, not yet responded.