ABSTRACT
As the key component of many hemoproteins (heme-containing proteins), heme is involved in a broad range of biological processes. Enzymes required for heme biosynthesis and degradation pathways are evolutionarily conserved. While heme metabolism has been studied extensively, the expression of heme metabolism enzymes during development has not been described. Here, we report that all heme biosynthases and two heme oxygenases, which initiate heme degradation, are dynamically expressed during Xenopus embryonic development. All heme synthases, with the exception of aminolevulinic acid synthase 2, are maternally expressed. At neurula stage, heme synthases are expressed in the developing neural tissue and in migrating neural crest cells. At the swimming tadpole stage, expression of heme synthases can be detected in multiple lineages, including eyes, neural crest cells, developing central nervous system, ventral blood island, pronephron, and pronephric tubule. Similar to heme synthases, heme oxygenases are expressed maternally. Zygotic expression of heme oxygenases is mainly restricted to the developing neural and neural crest lineages. Unlike heme synthases, heme oxygenases are not expressed in the ventral blood island and are expressed at a very low level in the pronephron and pronephric tubule. This indicates that heme metabolism may play important roles during development.
Subject(s)
Humans , Animals , Embryonic Development , Embryo, Nonmammalian/anatomy & histology , Embryo, Nonmammalian/physiology , Ferrochelatase/genetics , Ferrochelatase/metabolism , Gene Expression Regulation, Developmental , Heme/genetics , Heme/metabolism , Heme Oxygenase (Decyclizing)/genetics , Heme Oxygenase (Decyclizing)/metabolism , In Situ Hybridization , Xenopus Proteins/genetics , Xenopus Proteins/metabolism , Xenopus/embryology , Xenopus/genetics , Xenopus/metabolismABSTRACT
Se presenta paciente femenina de 27 años de edad, con antecedente de porfiria intermitente, la cual ingresa a la sala de partos de la Maternidad "Concepción Palacios" con embarazo simple de 33 semanas en trabajo de parto pre término. Al segundo día de puerperio presenta alza térmica, dolor abdominal, parestesia en miembros inferiores, orinas oscuras y trombocitopenia. Se diagnostica porfiria intermitente crónica en crisis, respondiendo al tratamiento indicado