Journal of Hematology & Thromboembolic Diseases

Journal of Hematology & Thromboembolic Diseases
Open Access

ISSN: 2329-8790

+44 1478 350008

Berkovitch-Luria G

  • Review Article
    ALA Induced Heme Synthesis: Fine Tuning Mechanisms of PBG Deaminase and ALA Dehydratase
    Author(s): Malik Zvi, Berkovitch-Luria G, Nudelman A and Rephaeli AMalik Zvi, Berkovitch-Luria G, Nudelman A and Rephaeli A

    During erythroid differentiation and maturation, three critical components α-globin, β-globin, and heme are critical for the formation of stable α2β2 hemoglobin (Hb) complexes. Heme synthesis enhances globin formation, not only because it is the precursor of Hb but also by modulating the machinery of gene expression and globin synthesis and coordinating erythroid cell maturation. Exogenous 5-aminolevulinic acid (ALA), the first precursor in the heme biosynthesis pathway, circumvents the rate limiting enzyme ALA synthase, and accelerates the heme synthesis pathway. ALA induced erythroid differentiation of human myelogenous leukemia K562 and murine erythroleukemia MEL cells, leads to hemoglobinization and cellular typical maturation. Only two key enzymes where known as regulator of the accumulation of PpIx: Porphobilinogen deaminase and Ferrochelatase. Recently we de.. View More»
    DOI: 10.4172/2329-8790.1000135

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