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

Abstract:

: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.

journal_name

Neurobiol Dis

journal_title

Neurobiology of disease

authors

Kiialainen A,Hovanes K,Paloneva J,Kopra O,Peltonen L

doi

10.1016/j.nbd.2004.09.007

keywords:

subject

Has Abstract

pub_date

2005-03-01 00:00:00

pages

314-22

issue

2

eissn

0969-9961

issn

1095-953X

pii

S0969-9961(04)00215-3

journal_volume

18

pub_type

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