Lysine degradation via formation of saccharopine is a pathway confined to the mitochondria. The second pathway for lysine degradation, the pipecolic acid pathway, is not yet fully elucidated and known enzymes are localized in the mitochondria, cytosol and peroxisome. The tissue-specific roles of these two pathways are still under investigation. The lysine degradation pathway is clinically relevant due to the occurrence of two severe neurometabolic disorders, pyridoxine-dependent epilepsy (PDE) and glutaric aciduria type 1 (GA1). The existence of three other disorders affecting lysine degradation without apparent clinical consequences opens up the possibility to find alternative therapeutic strategies for PDE and GA1 through pathway modulation. A better understanding of the mechanisms, compartmentalization and interplay between the different enzymes and metabolites involved in lysine degradation is of utmost importance.

通过形成糖精的赖氨酸降解是一种局限于线粒体的途径。赖氨酸降解的第二种途径，即胡椒酸途径，尚未完全阐明，已知的酶位于线粒体，胞质溶胶和过氧化物酶体中。这两种途径的组织特异性作用仍在研究中。由于发生两种严重的神经代谢疾病，吡ido醇依赖性癫痫病（PDE）和1型戊二酸尿症（GA1），赖氨酸降解途径在临床上具有相关性。其他三种影响赖氨酸降解而无明显临床后果的疾病的存在，为通过途径调节为PDE和GA1寻找替代治疗策略提供了可能性。更好地了解机制，