Serine代谢
中文名称
通路描述
L-丝氨酸在人类大脑中需求量很大，作为核苷酸、磷脂和神经递质甘氨酸和D-丝氨酸的重要前体。其合成途径始于3-磷酸甘油酸，随后需要谷氨酸作为氨基供体。参与该途径的酶缺乏会导致严重的神经系统症状，若早期治疗，使用丝氨酸可缓解症状（de Koning & Klomp 2004）。
英文描述
RHO GTPases regulate CFTR trafficking Activated RHO GTPase RHOQ (TC10) regulates the trafficking of CFTR (cystic fibrosis transmembrane conductance regulator) by binding to GOPC (Golgi-associated and PDZ and coiled-coil motif-containing protein) also known as PIST, FIG or CAL. GOPC is a Golgi resident protein that binds several membrane proteins, thereby modulating their expression. In the absence of RHOQ, GOPC bound to CFTR directs CFTR for lysosomal degradation, while GTP-bound RHOQ directs GOPC:CFTR complex to the plasma membrane, thereby rescuing CFTR (Neudauer et al. 2001, Cheng et al. 2005).
所含基因
3 个基因