Tiopronin

本公司产品仅用于科研实验，不得用于人体或动物的临床与诊断

硫普罗宁（商品名Thiola）是一种处方硫醇化合物，用于控制胱氨酸尿症疾病中胱氨酸沉淀和排泄的速率。

Tiopronin (trade name Thiola) is a prescription thiol drug used to control the rate of cystine precipitation and excretion in the disease cystinuria. Due to the rarity of the disorder, tioronin falls under the classification of an orphan drug.(In Vitro):In in vitro Von Hippel-Lindau protein binding assay, the inhibitory effect of Tiopronin (NMPG) on HPH-2 was attenuated by escalating dose of ascorbate but not 2-ketoglutarate, cofactors of the enzyme.(In Vivo):Tiopronin (NMPG) alleviates colonic injury and effectively lowered myeloperoxidase activity. Moreover, NMPG substantially attenuates expression of pro-inflammatory mediators in the inflamed colon. NMPG induces hypoxia-inducible factor-1α (HIF-1α) in human colon carcinoma cells, leading to elevated secretion of vascular endothelial growth factor (VEGF), a target gene product of HIF-1 involved in ulcer healing of gastrointestinal mucosa. NMPG induces HIF-1α occurred by inhibiting HIF prolyl hydroxylase-2 (HPH-2), an enzyme that plays a major role in negatively regulating HIF-1α protein stability.

In in vitro Von Hippel-Lindau protein binding assay, the inhibitory effect of Tiopronin (NMPG) on HPH-2 was attenuated by escalating dose of ascorbate but not 2-ketoglutarate, cofactors of the enzyme.

Tiopronin (NMPG) alleviates colonic injury and effectively lowered myeloperoxidase activity. Moreover, NMPG substantially attenuates expression of pro-inflammatory mediators in the inflamed colon. NMPG induces hypoxia-inducible factor-1α (HIF-1α) in human colon carcinoma cells, leading to elevated secretion of vascular endothelial growth factor (VEGF), a target gene product of HIF-1 involved in ulcer healing of gastrointestinal mucosa. NMPG induces HIF-1α occurred by inhibiting HIF prolyl hydroxylase-2 (HPH-2), an enzyme that plays a major role in negatively regulating HIF-1α protein stability.

BRN 1859822 | (±)-Tiopronin

Others

Other Targets

Others

Metabolic Disease

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1953-02-2

163.19

C5H9NO3S

>98% (HPLC)

Ethanol: 32 mg/mL (196.09 mM); Water: 32 mg/mL (196.09 mM); DMSO: 32 mg/mL (196.09 mM)

O=C(O)CNC(C(S)C)=O

Glycine, N-(2-mercapto-1-oxopropyl)- (9CI)

(-20℃)

With Ice Pack

≥ 2 years