LC3, an autophagosome marker, is expressed on oligodendrocytes in Nasu-Hakola disease brains.

ABSTRACT:

Background

Nasu-Hakola disease (NHD) is a rare autosomal recessive disorder characterized by sclerosing leukoencephalopathy and multifocal bone cysts, caused by a loss-of-function mutation of either DAP12 or TREM2. TREM2 and DAP12 constitute a receptor/adaptor signaling complex expressed exclusively on osteoclasts, dendritic cells, macrophages, and microglia. Neuropathologically, NHD exhibits profound loss of myelin and accumulation of axonal spheroids, accompanied by intense gliosis accentuated in the white matter of the frontal and temporal lobes. At present, the molecular mechanism responsible for development of leukoencephalopathy in NHD brains remains totally unknown.

Methods

By immunohistochemistry, we studied the expression of microtubule-associated protein 1 light ch

SUBMITTER: Satoh J 

PROVIDER: S-EPMC4022378 | biostudies-literature | 2014 May

REPOSITORIES: biostudies-literature