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  • Schematic Diagram Wilson'S Disease

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    Amira Charmaine
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    Wilson's disease is caused by a genetic defect on chromosome 13 that disrupts copper transport in the liver, causing copper to accumulate in tissues and organs over time. Normally, the liver regulates copper levels by incorporating copper into ceruloplasmin and secreting excess copper into bile, but in Wilson's disease this transport is disrupted, leading to a buildup of copper in the liver and other tissues that can cause oxidative damage and symptoms affecting the liver, brain, eyes, and other organs.

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    Wilson's disease

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    Schematic Diagram Pathophy

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    Wilson's disease is caused by a genetic defect on chromosome 13 that disrupts copper transport in the liver, causing copper to accumulate in tissues and organs over time. Normally, the liver regulates copper levels by incorporating copper into ceruloplasmin and secreting excess copper into bile, but in Wilson's disease this transport is disrupted, leading to a buildup of copper in the liver and other tissues that can cause oxidative damage and symptoms affecting the liver, brain, eyes, and other organs.

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    Attribution Non-Commercial (BY-NC)
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    48 views2 pages

    Schematic Diagram Wilson'S Disease

    Uploaded by

    Amira Charmaine
    Wilson's disease is caused by a genetic defect on chromosome 13 that disrupts copper transport in the liver, causing copper to accumulate in tissues and organs over time. Normally, the liver regulates copper levels by incorporating copper into ceruloplasmin and secreting excess copper into bile, but in Wilson's disease this transport is disrupted, leading to a buildup of copper in the liver and other tissues that can cause oxidative damage and symptoms affecting the liver, brain, eyes, and other organs.

    Copyright:

    Attribution Non-Commercial (BY-NC)
    Download as DOCX, PDF, TXT or read online from Scribd
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    Download as docx, pdf, or txt
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    SCHEMATIC DIAGRAM WILSONS DISEASE

    Cu enters body through digestive tract Disease genechromosome 13 Expressed primarily in liver, kidney, placenta

    Cu incorporated to ceruplasmin

    Increase Cu inside the cell

    Disruption of Cu transport into bile

    Normal physiology is disrupted

    Liver produces Cu into bloodstream not bound to ceruplasmin

    Enzyme (ATP7A) releases Cu into portal vein of liver

    Low serum Cu

    Free Cu circulates in the body

    Cu accumulates in liver tissue

    Paradoxical
    Precipitates in: Cu in liver overwhelm CHONs that normally bind it

    Increase urine Cu

    Kidneys Causes oxidative damage

    Kayser-Fleischer ring

    Eyes Chronic active hepatitis, fibrosis, cirrhosis

    Neuropsychiatric symptoms

    Brain

    Oxidation of Hgb, inhibition of energy-supplying enzymes in RBCs, direct damage to cell membrane

    NORMAL PATHWAY

    Increase Cu in cell

    Metallothionein bind Cu inside cell

    Excess Cu secreted into the bile

    Relsease of ceruplasmin in liver

    Ceruplasmin carries Cu into the bloodstream

    Normalized Cu levels and in the body

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