Rao V L, Qureshi I A, Butterworth R F
Neuroscience Research Unit, Hôpital St-Luc (University of Montreal), Quebec, Canada.
Pediatr Res. 1993 Dec;34(6):777-80. doi: 10.1203/00006450-199312000-00016.
Peripheral-type (mitochondrial) benzodiazepine receptors (PTBR) were studied in the brain and peripheral organs (kidney, liver, and testis) of normal male mice (CD-1/Y) and the congenitally hyperammonemic sparse fur (spf/Y) mouse. Radioligand binding assays were performed with [3H]PK 11195, a ligand with high selectivity and affinity for PTBR. Densities (maximal number of binding sites) of [3H]PK 11195 binding sites were greatest in kidney, followed by liver, testis, and brain. Densities of [3H]PK 11195 binding sites were significantly increased in all tissues of spf mice compared with control animals. In view of the localization of PTBR on the outer mitochondrial membrane, changes in PTBR in spf mouse tissues may modulate the altered mitochondrial function and oxidative metabolism, in brain and peripheral tissues, in congenital OTC deficiency. The positron emission tomography ligand 11C-PK 11195 could find an application in the assessment of end organ dysfunction in this disorder.
在正常雄性小鼠（CD - 1/Y）和先天性高氨血症稀毛（spf/Y）小鼠的脑及外周器官（肾、肝和睾丸）中研究了外周型（线粒体）苯二氮䓬受体（PTBR）。使用对PTBR具有高选择性和亲和力的配体[3H]PK 11195进行放射性配体结合测定。[3H]PK 11195结合位点的密度（结合位点的最大数量）在肾脏中最高，其次是肝脏、睾丸和脑。与对照动物相比，spf小鼠所有组织中[3H]PK 11195结合位点的密度均显著增加。鉴于PTBR定位于线粒体外膜，spf小鼠组织中PTBR的变化可能会调节先天性鸟氨酸转氨甲酰酶缺乏症患者脑和外周组织中线粒体功能和氧化代谢的改变。正电子发射断层扫描配体11C - PK 11195可用于评估该疾病终末器官功能障碍。
