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亮氨酸-rich重复激酶2(LRRK2)通过降低Rab7活性阻碍晚期内体出芽,从而延迟降解性受体运输。

LRRK2 delays degradative receptor trafficking by impeding late endosomal budding through decreasing Rab7 activity.

作者信息

Gómez-Suaga Patricia, Rivero-Ríos Pilar, Fdez Elena, Blanca Ramírez Marian, Ferrer Isidro, Aiastui Ana, López De Munain Adolfo, Hilfiker Sabine

机构信息

Institute of Parasitology and Biomedicine 'López-Neyra', Consejo Superior de Investigaciones Científicas (CSIC), Avda del Conocimiento s/n, 18016 Granada, Spain.

Institute of Neuropathology, IDIBELL-University Hospital Bellvitge, University of Barcelona, Llobregat, Spain.

出版信息

Hum Mol Genet. 2014 Dec 20;23(25):6779-96. doi: 10.1093/hmg/ddu395. Epub 2014 Jul 30.

Abstract

Mutations in the leucine-rich repeat kinase 2 (LRRK2) gene cause late-onset autosomal dominant Parkinson's disease (PD), and sequence variations at the LRRK2 locus are associated with increased risk for sporadic PD. LRRK2 contains both GTPase and kinase domains flanked by protein interaction motifs, and mutations associated with familial PD have been described for both catalytic domains. LRRK2 has been implicated in diverse cellular processes, and recent evidence pinpoints to an important role for LRRK2 in modulating a variety of intracellular membrane trafficking pathways. However, the underlying mechanisms are poorly understood. Here, by studying the classical, well-understood, degradative trafficking pathway of the epidermal growth factor receptor (EGFR), we show that LRRK2 regulates endocytic membrane trafficking in an Rab7-dependent manner. Mutant LRRK2 expression causes a slight delay in early-to-late endosomal trafficking, and a pronounced delay in trafficking out of late endosomes, which become aberrantly elongated into tubules. This is accompanied by a delay in EGFR degradation. The LRRK2-mediated deficits in EGFR trafficking and degradation can be reverted upon coexpression of active Rab7 and of a series of proteins involved in bridging the EGFR to Rab7 on late endosomes. Effector pulldown assays indicate that pathogenic LRRK2 decreases Rab7 activity both in cells overexpressing LRRK2, as well as in fibroblasts from pathogenic mutant LRRK2 PD patients when compared with healthy controls. Together, these findings provide novel insights into a previously unknown regulation of Rab7 activity by mutant LRRK2 which impairs membrane trafficking at very late stages of the endocytic pathway.

摘要

富含亮氨酸重复激酶2(LRRK2)基因的突变会导致晚发性常染色体显性帕金森病(PD),并且LRRK2基因座处的序列变异与散发性PD的风险增加相关。LRRK2包含由蛋白质相互作用基序侧翼的GTPase和激酶结构域,并且已经描述了与家族性PD相关的两个催化结构域的突变。LRRK2参与多种细胞过程,最近的证据表明LRRK2在调节各种细胞内膜运输途径中起重要作用。然而,其潜在机制尚不清楚。在这里,通过研究表皮生长因子受体(EGFR)经典的、已被充分理解的降解运输途径中,我们发现LRRK2以Rab7依赖的方式调节内吞膜运输。突变型LRRK2的表达导致早期至晚期内体运输略有延迟,以及从晚期内体运输的明显延迟,晚期内体会异常延长形成小管。这伴随着EGFR降解的延迟。在共表达活性Rab7和一系列参与将EGFR与晚期内体上的Rab7连接的蛋白质后,LRRK2介导的EGFR运输和降解缺陷可以得到恢复。效应子下拉试验表明,与健康对照相比,致病性LRRK2在过表达LRRK2的细胞中以及在致病性突变型LRRK2 PD患者的成纤维细胞中均降低了Rab7活性。总之,这些发现为突变型LRRK2对Rab7活性的前所未知的调节提供了新的见解,这种调节在内吞途径的非常晚期阶段损害膜运输。

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