Primate Unit and Delft Animal Centre, South African Medical Research Council, P.O Box 19070, Tygerberg, Cape Town, 7505, South Africa.
Medical Bioscience Department, University of the Western Cape, Belville, South Africa.
Metab Brain Dis. 2019 Oct;34(5):1467-1472. doi: 10.1007/s11011-019-00449-6. Epub 2019 Jun 22.
Nonketotic hyperglycinemia (NKH) is a neuro-metabolic disorder caused by a deficiency in the glycine cleavage system (GCS) and glycine transporter 1 (GlyT1). A case of atypical late onset of NKH has been reported in a colony of captive-bred Vervet monkeys. The purpose of this study was to evaluate the effect of sodium benzoate and dextromethorphan in reducing glycine levels in hyperglycinemic monkeys. Twelve captive-bred Vervet monkeys were assigned into three groups consisting of four animals (control, valproate induced and cataract with spontaneous hyperglycinemia). Valproate was used to elevate glycine levels and the induced group was then treated with sodium benzoate and dextromethorphan together with group three to normalise glycine levels in cerebrospinal fluid (CSF) and plasma. Valproate induction elicited changes in phosphate, alkaline phosphatase and platelet count, however, no significant changes in the glycine levels were observed, and this might be due to the individual variability within the group. The treatment intervention was only obtained in the spontaneous group whereby the glycine levels were normalised in CSF and plasma. Therefore, it can be concluded that sodium benzoate and dextromethorphan treatment was effective and beneficial to the hyperglycinemic group.
非酮症高甘氨酸血症(NKH)是一种由甘氨酸裂解系统(GCS)和甘氨酸转运蛋白 1(GlyT1)缺乏引起的神经代谢紊乱。据报道,在圈养繁殖的长尾猕猴群体中存在一例非典型迟发性 NKH 病例。本研究旨在评估苯甲酸钠和右美沙芬在降低高甘氨酸血症猴体内甘氨酸水平的效果。将 12 只圈养繁殖的长尾猕猴分为三组,每组 4 只(对照组、丙戊酸钠诱导组和白内障伴自发性高甘氨酸血症组)。丙戊酸钠用于升高甘氨酸水平,诱导组随后用苯甲酸钠和右美沙芬联合治疗,以将脑脊液(CSF)和血浆中的甘氨酸水平恢复正常。丙戊酸钠诱导引起了磷酸盐、碱性磷酸酶和血小板计数的变化,但甘氨酸水平没有明显变化,这可能是由于组内个体差异所致。只有在自发性组中获得了治疗干预,该组的 CSF 和血浆中的甘氨酸水平恢复正常。因此,可以得出结论,苯甲酸钠和右美沙芬治疗对高甘氨酸血症组是有效和有益的。