Defective MAOA does not oxidatively deaminate 5HT

Amine oxidase (flavin-containing) A (MAOA) catalyses the oxidative deamination of biogenic and dietary amines, the regulation of which is critical for mental state homeostasis. MAOA, located on the mitochondrial outer membrane and requiring FAD as cofactor, preferentially oxidises biogenic amines such as 5-hydroxytryptamine (5HT), dopamine, noradrenaline and adrenaline. Defects in MAOA can cause Brunner syndrome (BRUNS; MIM:300615), a form of X-linked non-dysmorphic mild mental retardation. Male patients are affected by mild mental retardation and exhibit abnormal behaviour, including impulsive aggression. A mutation that causes BRUNS is Q296* (Brunner et al. 1993).

胺氧化酶（含黄素）A（MAOA）催化生物发生和膳食胺的氧化脱氨作用，其对心理状态稳态的调节至关重要。位于线粒体外膜上的MAOA，需以FAD为辅因子，优先氧化生物胺，如5-羟色胺（5HT）、多巴胺、去甲肾上腺素和肾上腺素。MAOA的缺陷可导致布伦纳综合征（BRUNS；MIM:300615），一种X连锁的非典型轻度智力障碍。男性患者表现为轻度智力障碍和异常行为，包括冲动攻击性。导致BRUNS的突变是Q296*（布伦纳等，1993年）。