{"id":{"repo_id":"cape-town","oai_identifier":"oai:open.uct.ac.za:11427/3398"},"canonical_url":"https://search.dev.ndltd.org/etd/cape-town/oai:open.uct.ac.za:11427/3398","repository":{"repo_id":"cape-town","name":"University of Cape Town","base_url":"https://open.uct.ac.za/oai/request"},"display":{"title":"Variegate porphyria : molecular aspects of variegate porphyria in South Africa and their biochemical and clinical consequences","abstract":"Variegate porphyria (VP) is the clinical disorder associated with a deficiency of the haemsynthesising enzyme protoporphyrinogen oxidase (PPO). VP is one of the commonest monogenic inherited disorders in South Africa. The clinical effects include photocutaneous sensitivity and the development of potentially life-threatening acute porphyric crises. Section 1 of this dissertation examines the molecular basis for VP in South Africa.","abstract_html":"Variegate porphyria (VP) is the clinical disorder associated with a deficiency of the haemsynthesising enzyme protoporphyrinogen oxidase (PPO). VP is one of the commonest monogenic inherited disorders in South Africa. The clinical effects include photocutaneous sensitivity and the development of potentially life-threatening acute porphyric crises. 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VP is one of the commonest monogenic inherited disorders in South Africa. The clinical effects include photocutaneous sensitivity and the development of potentially life-threatening acute porphyric crises. Section 1 of this dissertation examines the molecular basis for VP in South Africa."]},{"key":"dc:title","label":"Title","values":["Variegate porphyria : molecular aspects of variegate porphyria in South Africa and their biochemical and clinical consequences"]}]}],"canonical_facts":{"dc:contributor.advisor":["Kirsch, Ralph"],"dc:creator":["Hift, R J"],"dc:date.accessioned":["2014-07-29T09:03:49Z"],"dc:date.available":["2014-07-29T09:03:49Z"],"dc:date.issued":["2000"],"dc:description":["Bibliography: p. 215-241."],"dc:description.abstract":["Variegate porphyria (VP) is the clinical disorder associated with a deficiency of the haemsynthesising enzyme protoporphyrinogen oxidase (PPO). VP is one of the commonest monogenic inherited disorders in South Africa. 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