| Sunday, July 29, 2001 |
| 2:00 PM - 11:00 PM | Arrival and Registration |
| 6:00 PM | Dinner |
| 7:30 PM - 9:30 PM | Molecular Structures and Protein Folding |
| (cochairs: Penny Handford and Hans Peter Bächinger) |
| Penny Handford (Oxford University)
Insights into fibrillin structure and function from domain studies |
| Glen Corson (Shriners Hospital for Children)
Fibrillin-3, a new addition to the family |
| Hans Peter Bächinger (Shriners Hospital for Children/OHSU)
The role of prolyl isomerase in protein folding |
| Tony Tamburro (Universita della Basilicata)
The molecular basis of elastin elasticity: the folded-extended structural dynamics |
| Elaine Davis (University of Texas Southwestern)
FKBP65: An ER proline isomerase that functions in organogenesis and tissue remodeling |
| 9:30 PM | Welcome Reception |
| Monday, July 30, 2001 |
| 7:30 AM - 8:30 AM | Breakfast |
| 8:45 AM | Conference Photo |
| 9:00 AM - 12:30 PM | Components of Elastic Fibers & Molecular Interactions |
| (cochairs: Joel Rosenbloom and Lynn Sakai) |
| Joel Rosenbloom (University of Pennsylvania)
Introduction |
| Alphonso Colombatti (University of Udine)
EMILIN-1 and the family of EMILIN proteins: structural and functional features |
| Mon-Li Chu (Thomas Jefferson University)
Fibulins: Molecular and Functional Analyses |
| Katalin Csiszar (University of Hawaii)
Complex interactions of lysyl oxidase and LOX-like proteins with nuclear factors and components of the extracellular matrix |
| Zenzo Isogai (Nagoya City University)
Versican and LTBP-1: Interactions with fibrillin and microfibrils |
| Fernando Segade (Washington University)
Update on MAGP-matrix binding domain and gene structure |
| 12:30 PM - 1:30 PM | Lunch |
| 4:30 PM - 6:00 PM | Posters |
| 6:00 PM | Dinner |
| 7:30 PM - 9:30 PM | Contributions of Elastic Fibers to Morphogenesis and Development |
| (cochairs: Francesco Ramirez and Mon-Li Chu) |
| Emilio Arteaga (Mt. Sinai Medical School)
Fibrillin-rich microfibrils: not just pretty macroaggregates |
| Bette Dzamba (University of Virginia)
Ectopic expression of the fourth 8-cysteine repeat of Fibrillin-2 in Xenopus laevis embryos suggests a role for fibrillin-2 in TGF beta superfamily signaling |
| Paolo Bonaldo (University of Padova)
Expression and functional analysis of emilin-1 in the mouse |
| Hiromi Yanagisawa (University of Texas Southwestern)
A new mouse model of elastic-fiber related disorder |
| Tomoyuki Nakamura (University of California at San Diego)
Dancing aorta: a new mouse model with disorganized elastic fibers |
| Tuesday, July 31, 2001 |
| 7:30 AM - 8:30 AM | Breakfast |
| 9:00 AM - 12:30 PM | LTBPs |
| (cochairs: Dan Rifkin and Sarah Dallas) |
| Dan Rifkin (New York University)
Introduction |
| Sarah Dallas (University of Manchester)
LTBP-1 assembly into the extracellular matrix |
| Jorma Keski-Oja (University of Helsinki)
New biological activities of latent TGFb binding protein domains |
| Kristy Downing (Oxford University)
Molecular basis for the interaction of LTBPs with LAP |
| Branka Dabovic (New York University)
LTBP-3 null mice |
| Harald von Melchner (University of Frankfurt Medical School)
Phenotypic consequences of a hypomorphic LTBP-4 allele in transgenic mice |
| 12:30 PM - 1:30 PM | Lunch |
| 4:30 PM - 6:00 PM | Posters |
| 6:00 PM | Dinner |
| 7:30 PM - 9:30 PM | Elastic Fibers and Cardiopulmonary Biology |
| (cochairs: Hal Dietz and Elaine Davis) |
| Hal Dietz (Johns Hopkins University)
Mouse models reveal novel pathogenetic mechanisms in the Marfan syndrome |
| Dean Li (University of Utah)
Molecular basis for distinguishing arteries from veins |
| Tom Wight (Hope Heart Institute)
Proteoglycans in the vascular system: regulators of elastic fiber and collagen assembly |
| Ian Hornstra (Washington University)
Underexpression of the murine lysyl oxidase (lox) gene is lethal at parturition from cardiovascular and diaphragmatic abnormalities |
| Wednesday, August 1, 2001 |
| 7:30 AM - 8:30 AM | Breakfast |
| 9:00 AM - 12:30 PM | Ultrastructure and Assembly of Elastic Fibers |
| (cochairs: Cay Kielty and Bob Mecham) |
| Bob Mecham (Washington University)
Quick freeze, deep etch three dimensional images of microfibrils |
| Cay Kielty (University of Manchester)
Fibrillin assembly and microfibril organization |
| Dieter Reinhardt (Medical University of Lübeck)
Interactions of fibrillin-1 with heparin/heparan sulfate: implications for microfibrillar assembly |
| Tim Ritty (Washington University)
So fibrillin can bind to heparin, but why would it want to? |
| Beth Kozel (Washington University)
Domains in tropoelastin important for association with microfibrils |
| 12:30 PM - 1:30 PM | Lunch |
| 4:30 PM - 6:00 PM | Posters |
| 6:00 PM | Dinner |
| 7:15 PM | Business Meeting |
| 7:30 PM - 9:30 PM | Diseases related to Elastic Fibers |
| (cochairs: Dianna Milewicz and Jeff Davidson) |
| Dianna Milewicz (University of Texas Houston)
Genetic and biochemical studies of fibrillin in scleroderma and other human diseases |
| Franziska Ringpfeil (Thomas Jefferson University)
Large deletion mutations and gene duplications in ABCC6: relevance to PXE |
| Charles Boyd (University of Hawaii)
Pathobiology of PXE |
| Jeff Davidson (Vanderbilt University)
SVAS and cutis laxa |
| Zsolt Urban (University of Hawaii)
A tandem duplication within the elastin gene is associated with autosomal dominant cutis laxa |
| Thursday, August 2, 2001 |
| 7:30 AM - 8:30 AM | Breakfast |
| 9:00 AM - 12:30 PM | Diverse Functions of Elastases and Matrix Fragments |
| (cochairs: Steve Shapiro and Carolyne Owen) |
| Steve Shapiro (Washington University)
Elastin fragments as mediators of inflammation |
| Sarah Dunsmore (University College London Medical School)
Not just NE old proteinase: new roles for neutrophil elastase in the lung |
| Carolyne Owen (University of Utah)
Neutrophil surface MMP-9: Focusing uninhibited elastolysis |
| Carole Wilson (Washington University)
Proteolytic processing of alpha-defensins by matrilysin (MMP-7) as a host defense mechanism |
| Pyong Park (Baylor College of Medicine)
Modulation of microbial pathogenesis by cell surface heparan sulfate proteoglycans |
| 12:30 PM - 1:30 PM | Lunch |
| 6:00 PM | Dinner |
| 7:30 PM - 9:30 PM | Mechanisms of Repair and Synthesis |
| (chair: Bill Parks) |
| Jeff Davidson (Vanderbilt University)
Cutis Laxa and elastin gene regulation |
| Celeste Rich (Boston University)
Transcriptional response of the elastin gene to matrix injury |
| Fred Keeley (Hospital for Sick Children)
Post-transcriptional regulation of elastin production |
| Rich Pierce (Washington University)
A novel XKLF-binding enhancer-like element in the first exon of the elastin gene |
