Dap12 and Trem2, molecules involved in innate immunity and neurodegeneration, are co-expressed in the CNS

Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL) is a recessively inherited disease characterized by early onset dementia associated with bone cysts. Our group has recently established the molecular background of PLOSL by identifying mutations in DAP12 and TREM2 g...

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Main Authors: Anna Kiialainen, Karine Hovanes, Juha Paloneva, Outi Kopra, Leena Peltonen
Format: Article
Language:English
Published: Elsevier 2005-03-01
Series:Neurobiology of Disease
Subjects:
Online Access:http://www.sciencedirect.com/science/article/pii/S0969996104002153
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author Anna Kiialainen
Karine Hovanes
Juha Paloneva
Outi Kopra
Leena Peltonen
author_facet Anna Kiialainen
Karine Hovanes
Juha Paloneva
Outi Kopra
Leena Peltonen
author_sort Anna Kiialainen
collection DOAJ
description Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL) is a recessively inherited disease characterized by early onset dementia associated with bone cysts. Our group has recently established the molecular background of PLOSL by identifying mutations in DAP12 and TREM2 genes. To understand how loss of function of the immune cell activating DAP12/TREM2 signaling complex leads to dementia and loss of myelin, we have analyzed here Dap12 and Trem2 expression in the mouse CNS. We show that Dap12 and Trem2 are expressed from embryonic stage to adulthood, and demonstrate a highly similar expression pattern. In addition, we identify microglial cells and oligodendrocytes as the major Dap12/Trem2-producing cells in the CNS and, consequently, as the predominant cell types involved in PLOSL pathogenesis. These findings provide a good starting point for the study of the molecular mechanisms of this inherited dementia and new evidence for the involvement of the immune system in neuronal degeneration.
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spelling doaj.art-10d9984a7e8d4d90b7ce0d7e22b55da52022-12-21T21:27:39ZengElsevierNeurobiology of Disease1095-953X2005-03-01182314322Dap12 and Trem2, molecules involved in innate immunity and neurodegeneration, are co-expressed in the CNSAnna Kiialainen0Karine Hovanes1Juha Paloneva2Outi Kopra3Leena Peltonen4Department of Molecular Medicine, National Public Health Institute, Helsinki, FinlandDepartment of Human Genetics, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USADepartment of Molecular Medicine, National Public Health Institute, Helsinki, FinlandDepartment of Molecular Medicine, National Public Health Institute, Helsinki, FinlandDepartment of Molecular Medicine, National Public Health Institute, Helsinki, Finland; Department of Human Genetics, David Geffen School of Medicine at UCLA, Los Angeles, CA 90095, USA; Corresponding author. National Public Health Institute, Department of Molecular Medicine, Biomedicum, Haartmaninkatu 8, 00290 Helsinki, Finland. Fax: +358 9 4744 8480.Polycystic lipomembranous osteodysplasia with sclerosing leukoencephalopathy (PLOSL) is a recessively inherited disease characterized by early onset dementia associated with bone cysts. Our group has recently established the molecular background of PLOSL by identifying mutations in DAP12 and TREM2 genes. To understand how loss of function of the immune cell activating DAP12/TREM2 signaling complex leads to dementia and loss of myelin, we have analyzed here Dap12 and Trem2 expression in the mouse CNS. We show that Dap12 and Trem2 are expressed from embryonic stage to adulthood, and demonstrate a highly similar expression pattern. In addition, we identify microglial cells and oligodendrocytes as the major Dap12/Trem2-producing cells in the CNS and, consequently, as the predominant cell types involved in PLOSL pathogenesis. These findings provide a good starting point for the study of the molecular mechanisms of this inherited dementia and new evidence for the involvement of the immune system in neuronal degeneration.http://www.sciencedirect.com/science/article/pii/S0969996104002153NeurodegenerationInnate immunityMicrogliaActivating receptorDAP12TREM2
spellingShingle Anna Kiialainen
Karine Hovanes
Juha Paloneva
Outi Kopra
Leena Peltonen
Dap12 and Trem2, molecules involved in innate immunity and neurodegeneration, are co-expressed in the CNS
Neurobiology of Disease
Neurodegeneration
Innate immunity
Microglia
Activating receptor
DAP12
TREM2
title Dap12 and Trem2, molecules involved in innate immunity and neurodegeneration, are co-expressed in the CNS
title_full Dap12 and Trem2, molecules involved in innate immunity and neurodegeneration, are co-expressed in the CNS
title_fullStr Dap12 and Trem2, molecules involved in innate immunity and neurodegeneration, are co-expressed in the CNS
title_full_unstemmed Dap12 and Trem2, molecules involved in innate immunity and neurodegeneration, are co-expressed in the CNS
title_short Dap12 and Trem2, molecules involved in innate immunity and neurodegeneration, are co-expressed in the CNS
title_sort dap12 and trem2 molecules involved in innate immunity and neurodegeneration are co expressed in the cns
topic Neurodegeneration
Innate immunity
Microglia
Activating receptor
DAP12
TREM2
url http://www.sciencedirect.com/science/article/pii/S0969996104002153
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