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Ned Porter studies an inborn error of metabolism, Smith-Lemli-Opitz syndrome (SLOS). The main metabolic defect, a reduced level of cholesterol and an increased level of dehydrocholesterol, primarily manifests itself with a range of brain abnormalities that include a combination of microcephaly, myelin maturation delay, lyssencephaly, agenesis/hypoplasia of corpus callosum, hypoplastic cerebellum, or other CNS deficits. Patients with SLOS also exhibit intellectual disability and autism-like symptoms.
Our preliminary findings and recently published data show that a) dehydrocholesterol (7-DHC) is, to our knowledge, the most reactive lipid toward free radical peroxidation that has been studied to date; b) the peroxidation products formed from 7-DHC can be understood based upon established chemical mechanisms; c) some of our newly discovered 7-DHC oxidation products are measurable both in a Dhcr7-deficient neuronal cells line and in the brain of Dhcr7-KO mice while these products are not present in control cells or mice; d) Dhcr7-KO mice exhibit higher levels of oxidative stress compared to controls; e) Dhcr7-deficient neuronal cells are susceptible to oxidative stress and lipid peroxidation initiated by peroxides or 6-hydroxydopamine; and f) pyridinol and pyrimidinol compounds constitute a new class of powerful antioxidants that we have discovered, and these compounds inhibit the formation of 7-DHC oxidation products in SLOS cells.
Porter’s current studies will lead to an understanding of the pathophysiological events underlying SLOS and move research toward the design of knowledge-based therapeutic approaches.
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NEUROPEDIATRICSno. 01 (2024): 23-31
Syed Masood, Hye-Young H. Kim, Edward R. Pennington, Keri A. Tallman,Ned A. Porter, Philip A. Bromberg,Rebecca L. Rice, Avram Gold,Zhenfa Zhang, James M. Samet
Redox Biology (2024): 103199
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Saori Noguchi, Keri A. Tallman,Ned A. Porter,Donald F. Stec,M. Wade Calcutt,William E. Boeglin,Alan R. Brash
A. Perryman, H. H. Kim,A. Payton,J. Rager, E. McNell, M. E. Rebuli, M. Almond, H. Wells, J. Antinori,N. E. Alexis,N. A. Porter, I. Jaspers
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2023)
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crossref(2023)
EUROPEAN RESPIRATORY JOURNAL (2023)
L. Leuenberger, K. A. Tallman, H. H. Kim, E. Schott, S. J. Cochran, M. B. Fessler,J. S. Bednash,J. C. Horowitz, S. Pannu,N. A. Porter, K. M. Gowdy
AMERICAN JOURNAL OF RESPIRATORY AND CRITICAL CARE MEDICINE (2023)
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