The invention features methods for the treatment of cystinosis and other cysteamine sensitive disorders in a subject including administration of a disulfide convertible to cysteamine in vivo. The methods can include the separate administration of a reducing agent to the subject to increase the bioavailablity and extend the plasma pharmacokinetic profile of the cysteamine produced following administration of the disulfide. The methods permit sustained cysteamine plasma concentrations in a subject.
这项发明涉及治疗囊性
氨基酸病和其他对半胱
氨酸敏感的疾病的方法,包括在体内给予可转化为半胱
氨酸的二
硫化物。该方法可以包括向受试者单独给予还原剂,以增加半胱
氨酸在给予二
硫化物后产生的
生物利用度,并延长血浆药代动力学特性。该方法允许受试者体内维持持续的半胱
氨酸血浆浓度。