Mutations in VPS13B, a member of a protein family implicated in bulk lipid transport between adjacent membranes, cause Cohen syndrome. VPS13B is known to be concentrated in the Golgi complex, but its precise location within this organelle and thus the site(s) where it achieves lipid transport remains unclear. Here, we show that VPS13B is localized at the interface between proximal and distal Golgi subcompartments and that Golgi complex reformation after Brefeldin A (BFA)-induced disruption is delayed in VPS13B KO cells. This delay is phenocopied by the loss of FAM177A1, a Golgi complex protein of unknown function reported to be a VPS13B interactor and whose mutations also result in a developmental disorder. In zebrafish, the vps13b ortholog, not previously annotated in this organism, genetically interacts with fam177a1. Collectively, these findings raise the possibility that bulk lipid transport by VPS13B may play a role in the dynamics of Golgi membranes and that VPS13B may be assisted in this function by FAM177A1.

中文翻译：

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VPS13B 位于高尔基体蓄水池之间的界面处，是 FAM177A1 的功能伙伴。


VPS13B 是蛋白质家族的一个成员，与相邻膜之间的大量脂质运输有关，其突变会导致 Cohen 综合征。已知 VPS13B 集中在高尔基复合体中，但其在该细胞器中的确切位置以及它实现脂质运输的位点仍不清楚。在这里，我们表明 VPS13B 位于近端和远端高尔基体亚区之间的界面，并且在 VPS13B KO 细胞中，布雷菲德菌素 A （BFA） 诱导的破坏后高尔基复合体重组延迟。这种延迟是通过 FAM177A1 的丢失进行表型复制的， 是一种功能未知的高尔基复合蛋白，据报道是 VPS13B 相互作用物，其突变也会导致发育障碍。在斑马鱼中，之前未在该生物体中注释的 vps13b 直系同源物与 fam177a1 发生遗传相互作用。总的来说，这些发现提出了 VPS13B 的大量脂质转运可能在高尔基体膜动力学中发挥作用的可能性，并且 VPS13B 可能FAM177A1协助此功能。