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OTHER MUTANTS

A: The following types of primary iron overload are associated with hepcidin:

Autosomal recessive:

1: Haemochromatosis associated with mutations in the HFE – gene in chromosome 6.

  • C282Y homozygous
  • C282Y/H63D compound heterozygous

2: Juvenile hereditary iron overload; mutation in the Hemojuvelin – gene in chromosome 1 or mutation in Hepcidin AntiMicrobial Peptide in chromosome 19.

  • mutation in HJV
  • mutation in HAMP

3: Hereditary iron overload; mutations in the TfR2 – gene in chromosome 7.


Autosomal dominant:

4: Hereditary iron overload; mutations in the Ferroportin (SLC11A3) – gene in chromosome 2.

Normal and HFE-mutant iron absorption


B: Other types of primary iron overload (rare diseases):

  • Aceruloplasminaemia
  • Congenital atransferrinaemia


Hereditary iron overload disorders in humans