Tmem106b (transmembrane protein 106B) encodes an endolysosomal membrane protein involved in myelination and dendrite morphogenesis. Overexpression or deletion in vitro influences lysosome size, position, and pH. TMEM106B has been identified as a risk modifier for several neurodegenerative diseases including frontotemporal lobar degeneration (FTLD-TDP), Alzheimer's disease, and hippocampal sclerosis. This strain was generated from embryonic stem cells provided by the International Knockout Mouse Consortium. Tmem106b knockout mice have exon 4 and the&nbsp; the lacZ/neomycin cassette deleted. Mice homozygous for the mutation are viable and fertile. When homozygous Tmem106b null mice are combined with the tauopathy model B6;C3-Tg(Prnp-MAPT*P301S)PS19Vle/J, double mutant progeny exhibit paralysis, decreased working and reference memory as well as a decline in short term memory by 6 months of age as compared to mice only carrying the tau mutation. Loss of TMEM106b in the tauopathy model accelerates accumulation of phosphorylated tau, accelerates neurological phenotypes, and worsens transcriptional signatures of disease in a mouse model of Alzheimer's/frontotemporal dementia.

Tmem106b knock-out mice have a deletion of exon 4 the Tmem106b (transmembrane protein 106B) gene. When combined with a mouse model of tauopathy (Tg(Prnp-MAPT*P301S)PS19Vle), double mutant mice exhibit an accelerated accumulation of phosphorylated tau and neurological phenotypes. This strain may be useful for studies of neurodegenerative diseases.

This strain is hosted by NIH's MMRRC, which partners with JAX for the distribution of this and other strains. For additional information and to purchase, please visit the MMRRC site.