Protein Misfolding and Misprocessing in Disease
Doubletree Hotel
Rockville, MD
May 4-5 2004
Summary
The National Institute of Diabetes & Digestive & Kidney Diseases (NIDDK) announces a meeting focusing on protein folding and misfolding, especially within the endoplasmic reticulum. There is extensive new research on this topic and on the mechanisms by which disease-associated proteins aggregate and injure cells. Speakers will address both basic science and clinical issues, and several will discuss new concepts for therapeutics, including the use of small molecules and chaperones to prevent protein misfolding or aggregation, to enhance the removal of toxic protein aggregates, or to block the cellular response to the defective protein.
Plenary talks, selected short talks, and a poster session will provide a forum for interactions among both basic and clinical scientists, as well as established and new investigators in the field.
Agenda
Tuesday, May 4, 2004
8:00 a.m. Registration and Continental Breakfast
8:30 a.m. Opening Remarks Allen Spiegel, MD Director, National Institute of Diabetes and Digestive and Kidney Diseases, National Institutes of HealthSteve Groft, PharmDDirector, Office of Rare Diseases, National Institutes of Health
8:40 a.m. Introductory AddressPeter Walter, PhD, University of California at San Francisco Signaling from the ER to the Nucleus
9:15 a.m. SESSION I
The ER as a Site for Protein Folding, Complex Assembly, and Quality Control for the Secretory Pathway
Chair: Dieter H. Wolf, PhD, Universitaet Stuttgart
- Hidde Ploegh, PhD, Harvard UniversityTBD
- Charlie Barlowe, PhD, Dartmouth UniversityTBD
- Dieter H. Wolf, PhD, Universitaet Stuttgart
Protein Quality Control in the Endoplasmic Reticulum and the Ubiquitin-proteasome-chaperone Connection
- Randy Hampton, PhD, University of California, San Diego Reversible, Small Molecule Regulation of ER Quality Control
10:45 a.m. Break
11:00 a.m. SESSION II
The Unfolded Protein Response: Maintenance of Proper Folding in the ER
Chair: David Ron, MD, New York University School of Medicine
- Doug Cavener, PhD, Pennsylvania State UniversityPERKs and the Unfolded Protein Response
- Linda Hendershot, PhD, St. Jude Children's Research Hospital
- ER Complexes and BIP Regulators
- David Ron, MD, New York University School of MedicineTranslational Control in the Unfolded Protein Response
- Jeff Brodsky, PhD, University of PittsburghMolecular Chaperone
- Action and Cellular Responses During ERAD
12:30 p.m. Lunch
2:00 p.m. Keynote Lecture William E. Balch, PhD, The Scripps Research InstituteProtein Traffic in Conformational Diseases
2:45 p.m. SESSION III ER Stress and Mis-folding
- Gert Kreibich, PhD, New York University School of MedicineMembrane Protein Assembly and Targeting in the Bladder Epithelium
- Craig B. Thompson, PhD, University of Pennsylvania Programmed Cell Death Induced by ER Stress • Sanjay Nigam, MD, University of California, San Diego
- Invited short presentations selected from abstracts
5:30 p.m. Poster Session/Reception
Wednesday, May 5, 2004
7:30 a.m. Continental Breakfast
8:00 a.m. Introductory Address Jeffrey W. Kelly, PhD, The Scripps Research InstituteAn Overview of Misfolding Diseases and Therapeutic Approaches
8:35 a.m. SESSION IV
Defective Protein Folding and Targeting in Renal Disease
Chair: Jeffrey W. Kelly, PhD, The Scripps Research Institute
- Michel Bouvier, PhD, Universite de Montreal Pharmacological Chaperones; A Potential Therapeutic Strategy for Nephrogenic Diabetes Insipidus and Other Conformational Diseases
- Chris Danpure, PhD, University College LondonPrimary Hyperoxaluria
- Peter Agre, MD, PhD, Johns Hopkins UniversityAquaporins
- Karl Tryggvason, MD, PhD, University of OuluFolding and Nephrin
10:05 a.m. Break
10:20 a.m. SESSION V
Mis-folding and Disease
Chair: David H. Perlmutter, MD, University of Pittsburgh School of Medicine
- David H. Perlmutter, MD, University of Pittsburgh School of MedicineAlpha 1 Antitrypsin Deficiency: Retention of an Aggregated Protein in ER Causes Liver Disease
- David Ellison, MD, Oregon Health & Science University Misfolding and ER Retention of Renal NCC in Gitelman’s Syndrome
- Show-Ling Shyng, PhD, Oregon Health & Science UniversityDefective Trafficking of K ATP Channels in Congenital Hyperinsulinism
- Invited short presentation selected from abstracts
11:50 AM Lunch 1:00 PM SESSION VI
Mis-folding and Disease
Chair: David H. Perlmutter, MD, University of Pittsburgh School of Medicine
- Jean Baum, PhD, Rutgers UniversityNMR and Folding Disease
- Dr. Laura S. Itzhaki, Hutchison/MRC Research CentreThe Role of Domain Swapping in Protein Aggregation
- Mark McNiven, PhD, Mayo ClinicDynamin and Endocytosis in Hepatocytes
2:10 p.m. Break
2:25 p.m. SESSION VII
Mis-folding, Disease and Therapeutic Approaches
Chair: Pam Zeitlin, MD, PhD, Johns Hopkins University
- Alan Verkman, MD, PhD, University of California at San FranciscoSmall Molecule Therapy and CF
- P. Michael Conn, PhD, Oregon Health & Science UniversityOrigami of the GnRH Receptor – A New Therapeutic Approach Unfolding
- Pam Zeitlin MD, PhD, Johns Hopkins School of MedicinePharmacologic Approaches to CFTR Misfolding
- Bob Desnick, MD, PhD, Mount Sinai School of MedicineChaperone-mediated Enzyme Enhancement Therapy
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