Dr. Herbert Bonkovsky has served with distinction on the APF Scientific Advisory Board since our founding, and is known by all patients who consult him in their care as a warm, compassionate and generous physician. He also has the distinction of having introduced another APF advisory board member, Dr. Steven Shedlofsky, to the study of the porphyrias.
Dr. Bonkovsky’s interest in the porphyrias began in medical school, and was strengthened during his time studying under porphyria researcher D.P. Tschudy at the National Institutes of Health. Dr. Bonkovsky was among the first to show that hepatic porphyrin and heme synthesis is under the negative feedback regulatory control of heme itself, acting chiefly to down-regulate delta-aminolevulinic acid (ALA) synthase-1, the rate controlling enzyme for heme synthesis. This discovery was the basis for developing heme therapy for acute porphyria attacks, which is still today the treatment of choice for these life-threatening attacks.
Since that time, Dr. Bonkovsky has continued the search for new treatments for the porphyrias, administering clinical trials for potential new therapies at hospitals and medical centers in Massachusetts, Connecticut, and now at the Wake Forest Baptist Medical Center in Winston-Salem, North Carolina.
Dr. Bonkovsky also identified deficiency of ferrochelatase, the final enzyme in the heme synthetic pathway as the fundamental metabolic defect in EPP. Studies on the regulation of hepatic heme metabolism, especially the mechanisms and factors that regulate ALA synthase and heme oxygenase, have been the subject of a large number of studies, both in the basic research laboratory and the clinical research center during the past 25 years. Dr. Bonkovsky has been fortunate to have had major RO1 grant funding from NIH (NIDDK) continually for the past quarter century to help support this research.