Chern Chian-Ru, Chern Chian-Jiang, Velíšková Jana, Velíšek Libor
Department of Cell Biology & Anatomy, New York Medical College School of Medicine, Valhalla, NY, 10595, USA.
Department of Cell Biology & Anatomy, New York Medical College School of Medicine, Valhalla, NY, 10595, USA; Department of Obstetrics & Gynecology, New York Medical College School of Medicine, Valhalla, NY, 10595, USA; Department of Neurology, New York Medical College School of Medicine, Valhalla, NY, 10595, USA.
Epilepsy Res. 2019 May;152:31-34. doi: 10.1016/j.eplepsyres.2019.03.004. Epub 2019 Mar 11.
Epileptic spasms during infancy (infantile spasms) represent a serious treatment and social problem despite their rare occurrence. Current treatments include hormonal therapy (adrenocorticotropin-ACTH or corticosteroids) or vigabatrin (per se or in the combination). These treatments are partially effective and with potentially significant adverse effects. Thus, the search for new effective drugs is warranted. We tested efficacy of a novel fusion peptide AQB-565 developed by Aequus Biopharma in a model of infantile spasms consisting of prenatal exposure to betamethasone and repeated postnatal trigger of spasms with N-methyl-d-aspartic acid (NMDA). AQB-565 molecule includes the first 24 amino acids of ACTH, a ten amino acid linker and a modified melanocyte-stimulating hormone molecule. In contrast to ACTH with almost uniform activity over all peripheral and central melanocortin receptor isoforms, AQB is preferentially active on central melanocortin receptors MC3 and MC4. Here, we used equivalent doses of rat ACTH (full molecule) and AQB-565 and compared their efficacy in a prospective randomized test against of repeated bouts of spasms on postnatal days (P)12, P13 and P15 in the rat model. All doses of ACTH (range 0.02-1.0 mg/kg s.c.) and all doses but one of AQB-565 in the same range suppressed spasms in P15 rats (treatment stopped on P14). There was no dose-dependent effect and both compounds had all-or-none effect that is similar to clinical outcome of hormonal treatment of infantile spasms in children. Thus, AQB-565 may represent a novel treatment of infantile spasms similarly effective as ACTH but with potentially limited side effects.
婴儿期癫痫性痉挛(婴儿痉挛症)尽管发生率很低,但却是一个严重的治疗和社会问题。目前的治疗方法包括激素疗法(促肾上腺皮质激素-ACTH或皮质类固醇)或氨己烯酸(单独使用或联合使用)。这些治疗方法部分有效,但可能有显著的不良反应。因此,有必要寻找新的有效药物。我们在一个婴儿痉挛症模型中测试了Aequus生物制药公司研发的新型融合肽AQB-565的疗效,该模型包括产前暴露于倍他米松以及产后用N-甲基-D-天冬氨酸(NMDA)反复诱发痉挛。AQB-565分子包含ACTH的前24个氨基酸、一个十肽连接子和一个修饰的促黑素细胞激素分子。与在所有外周和中枢黑素皮质素受体亚型上几乎具有均匀活性的ACTH不同,AQB优先作用于中枢黑素皮质素受体MC3和MC4。在此,我们使用等效剂量的大鼠ACTH(全分子)和AQB-565,并在一项前瞻性随机试验中比较了它们对大鼠模型出生后第(P)12、P13和P15天反复痉挛发作的疗效。所有剂量的ACTH(范围为0.02-1.0mg/kg皮下注射)以及相同范围内除一种剂量外的所有剂量的AQB-565都抑制了P15大鼠的痉挛(在P14停止治疗)。没有剂量依赖性效应,两种化合物都有全或无效应,这与儿童婴儿痉挛症激素治疗的临床结果相似。因此,AQB-565可能代表一种新型的婴儿痉挛症治疗方法,其效果与ACTH相似,但潜在副作用可能有限。