disease 1,786 words KG: ent-dise-0ec9037a 2026-03-22
kind:diseasesection:diseasesstate:published
Contents

Menkes Disease

Disease Info
Mitochondrial dysfunctionCytochrome c oxidase deficiency impairs oxidative phosphorylation, reducing cellular energy production, particularly in metabolically active neurons
Oxidative damageReduced superoxide dismutase activity leads to accumulation of oxidative-stress and oxidative damage to lipids, proteins, and DNA
Catecholamine imbalanceDopamine beta-hydroxylase deficiency disrupts catecholamine biosynthesis, with elevated DOPA-to-dihydroxyphenylglycol (DHPG) ratios serving as a diagnostic biomarker
DatabasesOMIMOrphanetClinicalTrialsPubMed

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