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This meeting took place in 2003



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Conformational Diseases of the Secretory Pathway (D2)


Organizer(s) Randal J. Kaufman and Tom A. Rapoport
March 1—6, 2003
Sagebrush Inn & Suites • Taos, New Mexico USA
Abstract Deadline: Nov 1, 2002
Late Abstract Deadline:
Scholarship Deadline:
Early Registration Deadline: Jan 3, 2003

Sponsored in part by The Director's Sponsor Fund

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.

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Conference Program    Print  |   View meeting in 12 hr (am/pm) time


SATURDAY, MARCH 1

15:00—19:00
Registration

Chamisa Lobby
18:15—19:15
Welcome

Chamisa Lobby
19:15—19:30
Orientation

Chamisa 1
19:30—20:30
Keynote Addresses
Meeting has ended...abstracts no longer viewable online.

Chamisa 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 2

07:00—08:00
Breakfast

Los Vaqueros
08:00—11:00
The Endoplasmic Reticulum
Meeting has ended...abstracts no longer viewable online.

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

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

Ari Helenius, ETH Zurich, Switzerland
Folding and Quality Control in the ER

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

09:20—09:40
Coffee Break

Chamisa Lobby
11:00—13:00
Poster Setup

Chamisa 2
13:00—22:00
Poster Viewing

Chamisa 2
16:30—17:00
Coffee & Snacks Available

Chamisa Lobby
17:00—19:00
The Contribution of N-Linked Glycans to Quality Control
Meeting has ended...abstracts no longer viewable online.

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

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

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

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

19:00—20:00
Social Hour

Chamisa 2
20:00—22:00
Poster Session 1

Chamisa 2

MONDAY, MARCH 3

07:00—08:00
Breakfast

Los Vaqueros
08:00—11:00
Disulfide Bridge Formation in the ER
Meeting has ended...abstracts no longer viewable online.

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

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

Ineke Braakman, Utrecht University, Netherlands
Folding and Disulfide Bond Formation in vitro and in vivo

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

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

09:20—09:40
Coffee Break

Chamisa Lobby
11:00—13:00
Poster Setup

Chamisa 2
13:00—22:00
Poster Viewing

Chamisa 2
14:30—16:30
Workshop 1: Protein Folding and Quality Control in the ER

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

* Linda M. Hendershot, St. Jude Children's Research Hospital, USA

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

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

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

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

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

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

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

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

16:30—17:00
Coffee & Snacks Available

Chamisa Lobby
17:00—19:00
Selective Folding and Export Processes
Meeting has ended...abstracts no longer viewable online.

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

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

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

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

19:00—20:00
Social Hour

Chamisa 2
20:00—22:00
Poster Session 2

Chamisa 2

TUESDAY, MARCH 4

07:00—08:00
Breakfast

Los Vaqueros
08:00—11:00
Proteolysis: ER-Associated Degradation and Death
Meeting has ended...abstracts no longer viewable online.

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

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

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

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

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

09:20—09:40
Coffee Break

Chamisa Lobby
11:00—13:00
Poster Setup

Chamisa 2
13:00—22:00
Poster Viewing

Chamisa 2
16:30—17:00
Coffee & Snacks Available

Chamisa Lobby
17:00—19:00
Extraction/Release of Polypeptides from Membranes
Meeting has ended...abstracts no longer viewable online.

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

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

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

19:00—20:00
Social Hour

Chamisa 2
20:00—22:00
Poster Session 3

Chamisa 2

WEDNESDAY, MARCH 5

07:00—08:00
Breakfast

Los Vaqueros
08:00—11:00
Cellular Response to Misfolded Protein
Meeting has ended...abstracts no longer viewable online.

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

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

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

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

09:20—09:40
Coffee Break

Chamisa Lobby
14:30—16:30
Workshop 2: ER Stress in Normal Development and Disease

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

* Linda M. Hendershot, St. Jude Children's Research Hospital, USA

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

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

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

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

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

Bo Feng, Columbia University, USA
Cholesterol Accumulation In Macrophages Induces the Unfolded Protein Response

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

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

16:30—17:00
Coffee & Snacks Available

Chamisa Lobby
17:00—19:00
Aberrant Protein Folding in Human Disease
Meeting has ended...abstracts no longer viewable online.

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

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

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

19:00—20:00
Social Hour

Chamisa 2
20:00—23:00
Entertainment

Chamisa 2

THURSDAY, MARCH 6

 
Departure


*Session Chair †Invited, not yet responded.



We gratefully acknowledge the generous grant for this conference provided by:


National Institutes of Health

Grant No. 1 R13 DK063935-01




We gratefully acknowledge additional support for this conference from:


Affinity BioReagents, Inc.

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