BEGIN:VCALENDAR VERSION:2.0 PRODID:-//WARF - ECPv5.16.4//NONSGML v1.0//EN CALSCALE:GREGORIAN METHOD:PUBLISH X-ORIGINAL-URL:https://www.warf.org X-WR-CALDESC:Events for WARF REFRESH-INTERVAL;VALUE=DURATION:PT1H X-Robots-Tag:noindex X-PUBLISHED-TTL:PT1H BEGIN:VTIMEZONE TZID:America/Chicago BEGIN:DAYLIGHT TZOFFSETFROM:-0600 TZOFFSETTO:-0500 TZNAME:CDT DTSTART:20230312T080000 END:DAYLIGHT BEGIN:STANDARD TZOFFSETFROM:-0500 TZOFFSETTO:-0600 TZNAME:CST DTSTART:20231105T070000 END:STANDARD END:VTIMEZONE BEGIN:VEVENT DTSTART;TZID=America/Chicago:20230428T113000 DTEND;TZID=America/Chicago:20230428T130000 DTSTAMP:20230803T045106 CREATED:20230301T144230Z LAST-MODIFIED:20230301T144230Z UID:29163-1682681400-1682686800@www.warf.org SUMMARY:Phenylketonuria: Insights into Pathophysiology and Novel Therapeutics DESCRIPTION:Phenylketonuria (PKU)\, due to recessively inherited phenylalanine hydroxylase (PAH) deficiency\, is among the most common inborn errors of metabolism with an incidence of 1:16\,000 live births in the US. PAH deficiency causes accumulation of the amino acid phenylalanine (Phe) to extremely high concentrations throughout the body but most importantly in brain. \nThe precise molecular mechanisms underpinning Phe’s effects upon the brain remain incompletely understood\, but left untreated\, chronic hyperphenylalaninemia causes severe global cognitive disability and seizures. For this reason\, all infants in the medically developed world are screened at birth for hyperphenylalaninemia and treated with a Phe-restricted diet. Although the diet prevents the major manifestations of the disease\, many shortcomings of contemporary dietary therapy drive the search for novel treatments. \nIn this lecture\, Dr. Cary Harding will review ongoing research efforts in animal models of PKU and in humans with PKU to understand the pathophysiologic effects of Phe. He will also discuss the development of novel therapies that are not strictly dependent upon dietary Phe restriction. These novel treatment approaches include enzyme substitution therapy which has gained FDA approval for use in adults with PKU\, liver-directed gene addition which is currently in clinical trials\, and preclinical investigation of gene editing in liver. \nHarding is a professor in the departments of molecular and medical genetics and pediatrics at Oregon Health & Science University (OHSU). He is board certified in pediatrics\, clinical genetics and biochemical genetics. He received his medical degree from the University of Washington followed by pediatric and medical genetics training at UW-Madison. \nHarding is a founding fellow of the American College of Medical Genetics. He is an attending physician on the OHSU clinical genetics service\, the clinical genetics clinic and the metabolic clinic at Doernbecher Children’s Hospital and the medical director of the biochemical genetics laboratory in the Knight Diagnostic Laboratories at OHSU. He is also a clinical consultant to the Northwest Regional Newborn Screening Program and to the state of Idaho Genetics Program. Harding’s basic and clinical research programs are focused upon the development of novel therapies\, including gene and cell therapies\, for inborn errors of metabolism. \nThis seminar is part of the Waisman Center John D. Wiley Seminar Series/WARF Therapeutics Drug Discovery Series. \nA luncheon will be held at 11:30 a.m.\, followed by the lecture at noon. URL:https://www.warf.org/event/phenylketonuria-insights-into-pathophysiology-and-novel-therapeutics/ LOCATION:John D. Wiley Conference Center\, Waisman Center\, 1500 Highland Ave.\, Madison\, WI\, 53705\, United States CATEGORIES:Event,Inventors, Entrepreneurs & Researchers,Other special events ATTACH;FMTTYPE=JPEG;ENCODING=BASE64;VALUE=BINARY:/9j/4QAYRXhpZgAASUkqAAgAAAAAAAAAAAAAAP/sABFEdWNreQABAAQAAABkAAD/4QONaHR0 cDovL25zLmFkb2JlLmNvbS94YXAvMS4wLwA8P3hwYWNrZXQgYmVnaW49Iu+7vyIgaWQ9Ilc1 TTBNcENlaGlIenJlU3pOVGN6a2M5ZCI/PiA8eDp4bXBtZXRhIHhtbG5zOng9ImFkb2JlOm5z Om1ldGEvIiB4OnhtcHRrPSJBZG9iZSBYTVAgQ29yZSA5LjAtYzAwMCA3OS5kYTRhN2U1ZWYs IDIwMjIvMTEvMjItMTM6NTA6MDcgICAgICAgICI+IDxyZGY6UkRGIHhtbG5zOnJkZj0iaHR0 cDovL3d3dy53My5vcmcvMTk5OS8wMi8yMi1yZGYtc3ludGF4LW5zIyI+IDxyZGY6RGVzY3Jp cHRpb24gcmRmOmFib3V0PSIiIHhtbG5zOnhtcE1NPSJodHRwOi8vbnMuYWRvYmUuY29tL3hh cC8xLjAvbW0vIiB4bWxuczpzdFJlZj0iaHR0cDovL25zLmFkb2JlLmNvbS94YXAvMS4wL3NU eXBlL1Jlc291cmNlUmVmIyIgeG1sbnM6eG1wPSJodHRwOi8vbnMuYWRvYmUuY29tL3hhcC8x LjAvIiB4bXBNTTpPcmlnaW5hbERvY3VtZW50SUQ9InhtcC5kaWQ6ZjhhODVlYmQtNzRhMS04 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