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缺乏 parkin 会导致猴模型中的神经退行性变和病理性 α-突触核蛋白的积累。

Deficiency of parkin causes neurodegeneration and accumulation of pathological α-synuclein in monkey models.

机构信息

State Key Laboratory of Bioactive Molecules and Druggability Assessment, Guangdong Key Laboratory of Non-human Primate Research, GHM Institute of CNS Regeneration, Jinan University, Guangzhou, China.

Hubei Topgene Biotechnological Research Institute Co., Ltd. Wuhan, China.

出版信息

J Clin Invest. 2024 Oct 15;134(20):e179633. doi: 10.1172/JCI179633.


DOI:10.1172/JCI179633
PMID:39403921
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11473153/
Abstract

Parkinson's disease (PD) is characterized by age-dependent neurodegeneration and the accumulation of toxic phosphorylated α-synuclein (pS129-α-syn). The mechanisms underlying these crucial pathological changes remain unclear. Mutations in parkin RBR E3 ubiquitin protein ligase (PARK2), the gene encoding parkin that is phosphorylated by PTEN-induced putative kinase 1 (PINK1) to participate in mitophagy, cause early onset PD. However, current parkin-KO mouse and pig models do not exhibit neurodegeneration. In the current study, we utilized CRISPR/Cas9 technology to establish parkin-deficient monkey models at different ages. We found that parkin deficiency leads to substantia nigra neurodegeneration in adult monkey brains and that parkin phosphorylation decreases with aging, primarily due to increased insolubility of parkin. Phosphorylated parkin is important for neuroprotection and the reduction of pS129-α-syn. Consistently, overexpression of WT parkin, but not a mutant form that cannot be phosphorylated by PINK1, reduced the accumulation of pS129-α-syn. These findings identify parkin phosphorylation as a key factor in PD pathogenesis and suggest it as a promising target for therapeutic interventions.

摘要

帕金森病(PD)的特征是年龄依赖性神经退行性变和毒性磷酸化α-突触核蛋白(pS129-α-syn)的积累。这些关键病理变化的机制尚不清楚。Parkin RBR E3 泛素蛋白连接酶(PARK2)基因的突变,该基因编码的 parkin 被磷酸化酶 1(PINK1)磷酸化参与线粒体自噬,导致早发性 PD。然而,目前的 parkin-KO 小鼠和猪模型并不表现出神经退行性变。在本研究中,我们利用 CRISPR/Cas9 技术在不同年龄建立 parkin 缺陷猴模型。我们发现 parkin 缺陷导致成年猴大脑黑质神经退行性变,并且 parkin 磷酸化随年龄增长而降低,主要是由于 parkin 的不溶性增加。磷酸化 parkin 对于神经保护和减少 pS129-α-syn 很重要。一致地,WT parkin 的过表达,而不是不能被 PINK1 磷酸化的突变形式,减少了 pS129-α-syn 的积累。这些发现确定了 parkin 磷酸化是 PD 发病机制中的关键因素,并表明它是治疗干预的有希望的靶点。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/0676745b5298/jci-134-179633-g077.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/ab70ef884dcb/jci-134-179633-g069.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/6af3756439cc/jci-134-179633-g070.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/b14910024cf3/jci-134-179633-g071.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/d0e7ffc2b446/jci-134-179633-g072.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/82b39990e5f1/jci-134-179633-g073.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/e96f3e098fd3/jci-134-179633-g074.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/df38dc5835b5/jci-134-179633-g075.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/32ec987d5dda/jci-134-179633-g076.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/0676745b5298/jci-134-179633-g077.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/ab70ef884dcb/jci-134-179633-g069.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/6af3756439cc/jci-134-179633-g070.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/b14910024cf3/jci-134-179633-g071.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/d0e7ffc2b446/jci-134-179633-g072.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/82b39990e5f1/jci-134-179633-g073.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/e96f3e098fd3/jci-134-179633-g074.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/df38dc5835b5/jci-134-179633-g075.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/32ec987d5dda/jci-134-179633-g076.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/bcea/11473153/0676745b5298/jci-134-179633-g077.jpg

相似文献

[1]
Deficiency of parkin causes neurodegeneration and accumulation of pathological α-synuclein in monkey models.

J Clin Invest. 2024-10-15

[2]
Effects of electroacupuncture on mitophagy mediated by SIRT3/PINK1/Parkin pathway in Parkinson's disease mice.

Zhen Ci Yan Jiu. 2024-3-25

[3]
Nigral overexpression of alpha-synuclein in the absence of parkin enhances alpha-synuclein phosphorylation but does not modulate dopaminergic neurodegeneration.

Mol Neurodegener. 2015-6-23

[4]
Convergence of Parkin, PINK1, and α-Synuclein on Stress-induced Mitochondrial Morphological Remodeling.

J Biol Chem. 2015-5-29

[5]
Nix restores mitophagy and mitochondrial function to protect against PINK1/Parkin-related Parkinson's disease.

Sci Rep. 2017-3-10

[6]
Bacterial artificial chromosome transgenic mice expressing a truncated mutant parkin exhibit age-dependent hypokinetic motor deficits, dopaminergic neuron degeneration, and accumulation of proteinase K-resistant alpha-synuclein.

J Neurosci. 2009-2-18

[7]
Enhanced Susceptibility of PINK1 Knockout Rats to α-Synuclein Fibrils.

Neuroscience. 2020-6-15

[8]
PTEN-Induced Putative Kinase 1 Dysfunction Accelerates Synucleinopathy.

J Parkinsons Dis. 2022

[9]
Inclusion body formation and neurodegeneration are parkin independent in a mouse model of alpha-synucleinopathy.

J Neurosci. 2006-4-5

[10]
Parkin depletion delays motor decline dose-dependently without overtly affecting neuropathology in α-synuclein transgenic mice.

BMC Neurosci. 2013-11-5

引用本文的文献

[1]
Urolithin A in Central Nervous System Disorders: Therapeutic Applications and Challenges.

Biomedicines. 2025-6-25

[2]
Formation of seeding-competent α-synuclein aggregates in parkin-deficient iPSC-derived human neurons.

NPJ Parkinsons Dis. 2025-6-21

[3]
PTEN: a new dawn in Parkinson's disease treatment.

Front Cell Neurosci. 2025-3-10

[4]
Bridging the gap: investigating the role of phosphorylation at the serine 129 site of α-synuclein in VAPB-PTPIP51 interactions.

Acta Neuropathol Commun. 2025-2-24

[5]
USP14 is crucial for proteostasis regulation and α-synuclein degradation in human SH-SY5Y dopaminergic cells.

Heliyon. 2025-1-23

[6]
Targeting Ferroptosis in Parkinson's Disease: Mechanisms and Emerging Therapeutic Strategies.

Int J Mol Sci. 2024-12-4

本文引用的文献

[1]
Differential distribution of PINK1 and Parkin in the primate brain implies distinct roles.

Neural Regen Res. 2025-4-1

[2]
Comparative analysis of primate and pig cells reveals primate-specific PINK1 expression and phosphorylation.

Zool Res. 2024-3-18

[3]
A primate nigrostriatal atlas of neuronal vulnerability and resilience in a model of Parkinson's disease.

Nat Commun. 2023-11-18

[4]
CHD8 mutations increase gliogenesis to enlarge brain size in the nonhuman primate.

Cell Discov. 2023-3-7

[5]
Alpha-synuclein in Parkinson's disease and other synucleinopathies: from overt neurodegeneration back to early synaptic dysfunction.

Cell Death Dis. 2023-3-1

[6]
H2A.X Phosphorylation in Oxidative Stress and Risk Assessment in Plasma Medicine.

Oxid Med Cell Longev. 2021

[7]
PINK1 kinase dysfunction triggers neurodegeneration in the primate brain without impacting mitochondrial homeostasis.

Protein Cell. 2022-1

[8]
Parkinson disease-associated cognitive impairment.

Nat Rev Dis Primers. 2021-7-1

[9]
The subcellular arrangement of alpha-synuclein proteoforms in the Parkinson's disease brain as revealed by multicolor STED microscopy.

Acta Neuropathol. 2021-9

[10]
Age-associated insolubility of parkin in human midbrain is linked to redox balance and sequestration of reactive dopamine metabolites.

Acta Neuropathol. 2021-5

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