disease 1,120 words KG: ent-dise-39a259d0 2026-03-22
kind:diseasesection:diseasesstate:published
Contents

Hereditary Hemochromatosis

Disease Info
C282Y (p.Cys282Tyr)The most frequent mutation, resulting in a cysteine-to-tyrosine substitution at position 282. Homozygotes for C282Y have significantly elevated transferrin saturation and ferritin levels.
H63D (p.His63Asp)A milder variant that may contribute to iron overload when compound heterozygous with C282Y or in combination with other genetic or environmental factors.
LiverHepatocytes and Kupffer cells
HeartCardiac myocytes
PancreasBeta cells of the Islets of Langerhans
Pituitary glandLeading to endocrine dysfunction
BrainBasal ganglia, substantia nigra, and cortical regions
Resting tremorTypically asymmetric
RigidityCogwheel-type rigidity
BradykinesiaSlowed movements
Postural instabilityBalance difficulties
DatabasesOMIMOrphanetClinicalTrialsPubMed

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