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      An Experimental Model of Neurodegenerative Disease Based on Porcine Hemagglutinating Encephalomyelitis Virus–Related Lysosomal Abnormalities

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          Abstract

          Lysosomes are involved in pathogenesis of a variety of neurodegenerative diseases and play a large role in neurodegenerative disorders caused by virus infection. However, whether virus-infected cells or animals can be used as experimental models of neurodegeneration in humans based on virus-related lysosomal dysfunction remain unclear. Porcine hemagglutinating encephalomyelitis virus displays neurotropism in mice, and neural cells are its targets for viral progression. PHEV infection was confirmed to be a risk factor for neurodegenerative diseases in the present. The findings demonstrated for the first time that PHEV infection can lead to lysosome disorders and showed that the specific mechanism of lysosome dysfunction is related to PGRN expression deficiency and indicated similar pathogenesis compared with human neurodegenerative diseases upon PHEV infection. Trehalose can also increase progranulin expression and rescue abnormalities in lysosomal structure in PHEV-infected cells. In conclusion, these results suggest that PHEV probably serve as a disease model for studying the pathogenic mechanisms and prevention of other degenerative diseases.

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          The online version of this article (10.1007/s12035-020-02105-y) contains supplementary material, which is available to authorized users.

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          Highly pathogenic H5N1 influenza virus can enter the central nervous system and induce neuroinflammation and neurodegeneration.

          Robert K. Shepherd, Katharine Sturm-Ramirez, Robert G. Webster … (2009)
          One of the greatest influenza pandemic threats at this time is posed by the highly pathogenic H5N1 avian influenza viruses. To date, 61% of the 433 known human cases of H5N1 infection have proved fatal. Animals infected by H5N1 viruses have demonstrated acute neurological signs ranging from mild encephalitis to motor disturbances to coma. However, no studies have examined the longer-term neurologic consequences of H5N1 infection among surviving hosts. Using the C57BL/6J mouse, a mouse strain that can be infected by the A/Vietnam/1203/04 H5N1 virus without adaptation, we show that this virus travels from the peripheral nervous system into the CNS to higher levels of the neuroaxis. In regions infected by H5N1 virus, we observe activation of microglia and alpha-synuclein phosphorylation and aggregation that persists long after resolution of the infection. We also observe a significant loss of dopaminergic neurons in the substantia nigra pars compacta 60 days after infection. Our results suggest that a pandemic H5N1 pathogen, or other neurotropic influenza virus, could initiate CNS disorders of protein aggregation including Parkinson's and Alzheimer's diseases.
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            Animal models of neurodegenerative diseases

            Ted Dawson, Todd E. Golde, Clotilde Lagier-Tourenne (2018)
            Animal models of adult-onset neurodegenerative diseases have enhanced the understanding of the molecular pathogenesis of Alzheimer’s disease (AD), Parkinson’s disease (PD), frontotemporal dementia (FTD) and amyotrophic lateral sclerosis (ALS). Nevertheless, our understanding of these disorders and the development of mechanistically designed therapeutics can still benefit from more rigorous use of the models and from the generation of animals that more faithfully recapitulate human disease. Here we review the current state of rodent models for AD, PD, FTD and ALS. We discuss limitations and utility of current models, issues regarding translatability, and future directions for developing animal models of these human disorders.
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              The granulin gene family: from cancer to dementia.

              Andrew Bateman, Tom J H Bennett (2009)
              The growth factor progranulin (PGRN) regulates cell division, survival, and migration. PGRN is an extracellular glycoprotein bearing multiple copies of the cysteine-rich granulin motif. With PGRN family members in plants and slime mold, it represents one of the most ancient of the extracellular regulatory proteins still extant in modern animals. PRGN has multiple biological roles. It contributes to the regulation of early embryogenesis, to adult tissue repair and inflammation. Elevated PGRN levels often occur in cancers, and PGRN immunotherapy inhibits the growth of hepatic cancer xenografts in mice. Recent studies have demonstrated roles for PGRN in neurobiology. An autosomal dominant mutation in GRN, the gene for PGRN, leads to neuronal atrophy in the frontal and temporal lobes, resulting in the disease frontotemporal lobar dementia. In this review we will discuss current knowledge of the multifaceted biology of PGRN.
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                Author and article information

                Contributors
                Wenqi He:
                ORCID: http://orcid.org/0000-0003-3254-1242
                hewq@jlu.edu.cn
                Journal
                Journal ID (nlm-ta): Mol Neurobiol
                Journal ID (iso-abbrev): Mol. Neurobiol
                Title: Molecular Neurobiology
                Publisher: Springer US (New York )
                ISSN (Print): 0893-7648
                ISSN (Electronic): 1559-1182
                Publication date (Electronic): 2 September 2020
                Pages: 1-8
                Affiliations
                [1 ]GRID grid.64924.3d, ISNI 0000 0004 1760 5735, Key Laboratory of Zoonosis Research, Ministry of Education, College of Veterinary Medicine, , Jilin University, ; Changchun, China
                [2 ]Jilin Academy of Animal Husbandry and Veterinary Medicine, Changchun, Jilin China
                [3 ]GRID grid.430605.4, Department of Neurosurgery, , The First Hospital of Jilin University, ; Changchun, 130021 China
                Author information
                http://orcid.org/0000-0003-3254-1242
                Article
                Publisher ID: 2105
                DOI: 10.1007/s12035-020-02105-y
                PMC ID: 7463228
                PubMed ID: 32876841
                SO-VID: 23033ebc-e59a-4182-96e6-ca30d14bb7f0
                Copyright © © Springer Science+Business Media, LLC, part of Springer Nature 2020
                License:

                This article is made available via the PMC Open Access Subset for unrestricted research re-use and secondary analysis in any form or by any means with acknowledgement of the original source. These permissions are granted for the duration of the World Health Organization (WHO) declaration of COVID-19 as a global pandemic.

                History
                Date received : 19 May 2020
                Date accepted : 27 August 2020
                Funding
                Funded by: the National Key Research and Development Program of China
                Award ID: 2016YFD0500102
                Award Recipient :
                Funded by: National Natural Science Foundation of China
                Award ID: 31902262
                Award Recipient :
                Funded by: FundRef http://dx.doi.org/10.13039/501100001809, National Natural Science Foundation of China;
                Award ID: 31872446
                Award ID: 31772704
                Award Recipient :
                Categories
                Subject: Original Article

                ScienceOpen disciplines: Neurosciences
                Keywords: neurodegenerative diseases,porcine hemagglutinating encephalomyelitis virus,lysosomal abnormalities,progranulin,trehalose
                Data availability:
                ScienceOpen disciplines: Neurosciences
                Keywords: neurodegenerative diseases, porcine hemagglutinating encephalomyelitis virus, lysosomal abnormalities, progranulin, trehalose

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