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Erythropoietic Protoporphyria and X-Linked Protoporphyria: Pathophysiology, Genetics, Clinical Manifestations, and Management

Erythropoietic Protoporphyria (EPP) and X-linked Protoporphyria (XLP) are rare, genetic photodermatoses resulting from defects in enzymes of the heme-biosynthetic pathway. EPP results from the partial deficiency of ferrochelatase, and XLP results from gain-of-function mutations in erythroid specific...

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Wedi'i Gadw mewn:
Manylion Llyfryddiaeth
Cyhoeddwyd yn:Mol Genet Metab
Prif Awdur: Balwani, Manisha
Fformat: Artigo
Iaith:Inglês
Cyhoeddwyd: 2019
Pynciau:
Mynediad Ar-lein:https://ncbi.nlm.nih.gov/pmc/articles/PMC6656624/
https://ncbi.nlm.nih.gov/pubmed/30704898
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.ymgme.2019.01.020
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