Brunner syndrome


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Brunner syndrome

A form of X-linked, non-dysmorphic, mild mental retardation (OMIM:300615), in which affected males have borderline mental retardation and abnormal behaviour, including poor impulse control. Female carriers have normal intelligence and behaviour.

Molecular pathology
Caused by defects of MAOA, which encodes a mitochondrial enzyme that catalyses the oxidative deamination of biogenic (e.g., dopamine, norepinephrine, serotonin and other neurotransmitters) and xenobiotic amines.
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