Trans-L-3-hydroxyproline dehydratase (L3HYPDH)

**Trans-L-3-hydroxyproline dehydratase (L3HYPDH)** is an enzyme that specifically catalyzes the dehydration of trans-3-hydroxy-L-proline to delta-1-pyrroline-2-carboxylate (Pyr2C)[9][5][2]. This reaction is a critical step in the metabolism and degradation of trans-3-hydroxy-L-proline, an amino acid derivative found primarily in collagen—especially collagen IV, a structural component in basement membranes[1][2][3][5]. The human enzyme is encoded by the *L3HYPDH* gene, previously known as C14orf149, and is structurally and evolutionarily related to proline racemase-like enzymes[1][4]. L3HYPDH appears important for the catabolism of trans-3-hydroxy-L-proline taken from diet or released during normal protein turnover, ensuring the proper maintenance of amino acid balance and collagen homeostasis[1][2][9]. While no drugs are currently known to target this enzyme, and there are no established disease associations or biomarker uses, disturbances in this pathway could be implicated in connective tissue or basement membrane-related diseases.

Other names

trans-3-hydroxy-L-proline dehydratasetrans-L-3-hydroxyproline hydro-lyase (Δ1-pyrroline-2-carboxylate-forming)T3HPDC14orf149 (human gene)

Not applicable (no drugs described to act via this enzyme), but for physiological function: catalyzes dehydration of trans-3-hydroxy-L-proline to delta-1-pyrroline-2-carboxylate