Mouad Ait Kbaich, Jennifer L Johnson, Sergio D Catz
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引用次数: 0
摘要
RAB11FIP4 （RAB11 family interacting protein 4）是在胱氨酸病中下调的RAB11A （Ras-related protein raba -11）效应蛋白，在细胞运输中起重要作用。RAB11FIP4在胱氨酸细胞中的重构可恢复多种细胞功能，包括溶酶体运输、自噬和内质网应激反应。这些发现表明RAB11FIP4既是胱氨酸病发病机制的关键参与者，也是一个有希望的治疗靶点。这篇文章的目的是强调恢复RAB11FIP4的表达是如何通过调节转运途径来恢复胱氨酸病的细胞稳态的。
Untangling Traffic Jams: RAB11FIP4 Orchestrates Cellular Recovery in Cystinosis.
RAB11FIP4 (RAB11 family interacting protein 4), a RAB11A (Ras-related protein Rab-11) effector protein downregulated in cystinosis, plays a crucial role in cellular trafficking. Reconstitution of RAB11FIP4 in cystinotic cells restores multiple cellular functions, including lysosomal trafficking, autophagy, and the endoplasmic reticulum stress response. These findings identify RAB11FIP4 as both a key player in cystinosis pathogenesis and a promising therapeutic target. The purpose of this punctum is to highlight how restoring RAB11FIP4 expression rescues cellular homeostasis in cystinosis through the regulation of trafficking pathways.
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