Filippini Alice, Carini Giulia, Barbon Alessandro, Gennarelli Massimo, Russo Isabella
IRCCS Istituto Centro San Giovanni di Dio Fatebenefratelli, Brescia, Italy.
Unit of Biology and Genetics, Department of Molecular and Translational Medicine, University of Brescia, Brescia, Italy.
Acta Neuropathol Commun. 2025 May 12;13(1):98. doi: 10.1186/s40478-025-02015-x.
Accumulating evidence highlights that dysfunction of astrocyte biology might contribute to Parkinson's disease (PD) onset and progression. Leucine-rich repeat kinase 2 (LRRK2), a gene linked to genetic and familial PD, has been reported to affect astrocytic-related functions, including the ingestion of alpha-synuclein (α-syn) aggregates. In this context, we recently showed that the extracellular chaperone clusterin (Clu) binds to and limits the uptake of alpha-syn fibrils by astrocytes. Thus, starting from these premises, we explored whether LRRK2 G2019S affects aggregated α-syn ingestion through the Clu-related pathway and the underlying molecular mechanisms. We first validated in our LRRK2 G2019S knock-in (KI) mouse strain that primary astrocytes exhibited an impaired ability to ingest fibrillary α-syn. Then, we investigated whether LRRK2 G2019S affects this pathway through the modulation of Clu. In this regard, we collected several results showing that LRRK2 regulates Clu levels in astrocytes. Specifically, brain slices and primary astrocytes from KI mice with the LRRK2 G2019S pathological mutation exhibit increased levels of Clu protein compared to their respective wild-type (WT). Accordingly, we observed an opposite effect in brain slices and primary astrocytes from LRRK2 knock-out (KO) mice in comparison to their respective WT. To gain insights into the molecular mechanism underlying LRRK2-dependent Clu modulation, we found that LRRK2 controls Clu expression at the translation level through the action of miR-22-5p. In addition, we demonstrated that treatment with miR-22-5p mimic improves the ability of LRRK2 G2019S-KI astrocytes to take up α-syn pffs. Taken together, our findings indicate that the LRRK2-Clu pathway is involved in the ingestion of a-syn fibrils and that the impairment of α-syn uptake in LRRK2 G2019S-KI astrocytes is associated to Clu levels. Future studies will allow us to understand whether the modulation of astrocytic LRRK2 G2019S-Clu pathway might attenuate the neuronal spreading of α-syn pathology in PD.
越来越多的证据表明,星形胶质细胞生物学功能障碍可能与帕金森病(PD)的发病和进展有关。富含亮氨酸重复激酶2(LRRK2)是一种与遗传性和家族性PD相关的基因,据报道它会影响与星形胶质细胞相关的功能,包括摄取α-突触核蛋白(α-syn)聚集体。在此背景下,我们最近发现细胞外伴侣蛋白簇集素(Clu)能结合并限制星形胶质细胞对α-syn纤维的摄取。因此,基于这些前提,我们探究了LRRK2 G2019S是否通过与Clu相关的途径影响聚集的α-syn摄取及其潜在的分子机制。我们首先在我们的LRRK2 G2019S基因敲入(KI)小鼠品系中验证了原代星形胶质细胞摄取纤维状α-syn的能力受损。然后,我们研究了LRRK2 G2019S是否通过调节Clu来影响这一途径。在这方面,我们收集了多项结果,表明LRRK2调节星形胶质细胞中Clu的水平。具体而言,与各自的野生型(WT)相比,携带LRRK2 G2019S病理突变的KI小鼠的脑切片和原代星形胶质细胞中Clu蛋白水平升高。相应地,与各自的WT相比,我们在LRRK2基因敲除(KO)小鼠的脑切片和原代星形胶质细胞中观察到相反的效果。为了深入了解LRRK2依赖性Clu调节的分子机制,我们发现LRRK2通过miR-22-5p的作用在翻译水平上控制Clu的表达。此外,我们证明用miR-22-5p模拟物处理可提高LRRK2 G2019S-KI星形胶质细胞摄取α-syn原纤维的能力。综上所述,我们的研究结果表明LRRK2-Clu途径参与了α-syn纤维的摄取,并且LRRK2 G2019S-KI星形胶质细胞中α-syn摄取的受损与Clu水平有关。未来的研究将使我们了解调节星形胶质细胞LRRK2 G2019S-Clu途径是否可能减弱PD中α-syn病理的神经元传播。