Zycubo (copper histidinate), the first treatment for pediatric Menkes disease

Zycubo(组氨酸铜)是治疗儿童门克斯病的首个药物。

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Abstract

Copper histidinate (marketed as ZYCUVO(®), formerly known as CUTX-101) is a copper replacement product indicated for the treatment of Menkes disease in pediatric patients. The chemical name is copper, (L-histidinato-κN,κN3,κO)(L-histidinato-κN,κO)-, (SP-5–14-C)- or copper bis((2S)-2-amino-3-(1H-imidazol-5-yl)propanoate). Copper histidinate has a molecular formula of C(12)H(16)CuN(6)O(4) and a molecular weight of 371.84 g/mol. The molecule consists of a central copper (II) ion coordinated by two L-histidine ligands through nitrogen and oxygen donor atoms, forming a stable chelated complex that facilitates systemic copper delivery following subcutaneous administration. Copper histidinate is fully soluble in water, forming a blue solution. Reconstituted solutions must be used within 4 hours at room temperature (20°C to 25°C) or within 24 hours if refrigerated (2°C to 8°C).

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