The regulation of cellular iron metabolism

A.C.G. Chua, Ross Graham, Debbie Trinder, John Olynyk

    Research output: Contribution to journalArticle

    113 Citations (Scopus)

    Abstract

    While iron is an essential trace element required by nearly all living organisms, deficiencies or excesses can lead to pathological conditions such as iron deficiency anemia or hemochromatosis, respectively. A decade has passed since the discovery of the hemochromatosis gene, HFE, and our understanding of hereditary hemochromatosis (HH) and iron metabolism in health and a variety of diseases has progressed considerably. Although HFE-related hemochromatosis is the most widespread, other forms of HH have subsequently been identified. These forms are not attributed to mutations in the HFE gene but rather to mutations in genes involved in the transport, storage, and regulation of iron. This review is an overview of cellular iron metabolism and regulation, describing the function of key proteins involved in these processes, with particular emphasis on the liver's role in iron homeostasis, as it is the main target of iron deposition in pathological iron overload. Current knowledge on their roles in maintaining iron homeostasis and how their dysregulation leads to the pathogenesis of HH are discussed.
    Original languageEnglish
    Pages (from-to)413-459
    JournalCritical Reviews in Clinical Laboratory Sciences
    Volume44
    Issue number5-6
    DOIs
    Publication statusPublished - 2007

    Fingerprint

    Hemochromatosis
    Metabolism
    Iron
    Genes
    Homeostasis
    Mutation
    Iron Overload
    Iron-Deficiency Anemias
    Trace Elements
    Genetic Association Studies
    Liver
    Health
    Proteins

    Cite this

    @article{24bb870e7f8c44d8bd8e4ba0d66d89c2,
    title = "The regulation of cellular iron metabolism",
    abstract = "While iron is an essential trace element required by nearly all living organisms, deficiencies or excesses can lead to pathological conditions such as iron deficiency anemia or hemochromatosis, respectively. A decade has passed since the discovery of the hemochromatosis gene, HFE, and our understanding of hereditary hemochromatosis (HH) and iron metabolism in health and a variety of diseases has progressed considerably. Although HFE-related hemochromatosis is the most widespread, other forms of HH have subsequently been identified. These forms are not attributed to mutations in the HFE gene but rather to mutations in genes involved in the transport, storage, and regulation of iron. This review is an overview of cellular iron metabolism and regulation, describing the function of key proteins involved in these processes, with particular emphasis on the liver's role in iron homeostasis, as it is the main target of iron deposition in pathological iron overload. Current knowledge on their roles in maintaining iron homeostasis and how their dysregulation leads to the pathogenesis of HH are discussed.",
    author = "A.C.G. Chua and Ross Graham and Debbie Trinder and John Olynyk",
    year = "2007",
    doi = "10.1080/10408360701428257",
    language = "English",
    volume = "44",
    pages = "413--459",
    journal = "Critical Reviews in Clinical Laboratory Sciences",
    issn = "1040-8363",
    publisher = "Informa Healthcare USA",
    number = "5-6",

    }

    The regulation of cellular iron metabolism. / Chua, A.C.G.; Graham, Ross; Trinder, Debbie; Olynyk, John.

    In: Critical Reviews in Clinical Laboratory Sciences, Vol. 44, No. 5-6, 2007, p. 413-459.

    Research output: Contribution to journalArticle

    TY - JOUR

    T1 - The regulation of cellular iron metabolism

    AU - Chua, A.C.G.

    AU - Graham, Ross

    AU - Trinder, Debbie

    AU - Olynyk, John

    PY - 2007

    Y1 - 2007

    N2 - While iron is an essential trace element required by nearly all living organisms, deficiencies or excesses can lead to pathological conditions such as iron deficiency anemia or hemochromatosis, respectively. A decade has passed since the discovery of the hemochromatosis gene, HFE, and our understanding of hereditary hemochromatosis (HH) and iron metabolism in health and a variety of diseases has progressed considerably. Although HFE-related hemochromatosis is the most widespread, other forms of HH have subsequently been identified. These forms are not attributed to mutations in the HFE gene but rather to mutations in genes involved in the transport, storage, and regulation of iron. This review is an overview of cellular iron metabolism and regulation, describing the function of key proteins involved in these processes, with particular emphasis on the liver's role in iron homeostasis, as it is the main target of iron deposition in pathological iron overload. Current knowledge on their roles in maintaining iron homeostasis and how their dysregulation leads to the pathogenesis of HH are discussed.

    AB - While iron is an essential trace element required by nearly all living organisms, deficiencies or excesses can lead to pathological conditions such as iron deficiency anemia or hemochromatosis, respectively. A decade has passed since the discovery of the hemochromatosis gene, HFE, and our understanding of hereditary hemochromatosis (HH) and iron metabolism in health and a variety of diseases has progressed considerably. Although HFE-related hemochromatosis is the most widespread, other forms of HH have subsequently been identified. These forms are not attributed to mutations in the HFE gene but rather to mutations in genes involved in the transport, storage, and regulation of iron. This review is an overview of cellular iron metabolism and regulation, describing the function of key proteins involved in these processes, with particular emphasis on the liver's role in iron homeostasis, as it is the main target of iron deposition in pathological iron overload. Current knowledge on their roles in maintaining iron homeostasis and how their dysregulation leads to the pathogenesis of HH are discussed.

    U2 - 10.1080/10408360701428257

    DO - 10.1080/10408360701428257

    M3 - Article

    VL - 44

    SP - 413

    EP - 459

    JO - Critical Reviews in Clinical Laboratory Sciences

    JF - Critical Reviews in Clinical Laboratory Sciences

    SN - 1040-8363

    IS - 5-6

    ER -