Topic: lysosomal membrane protein

CLN3 is a lysosomal membrane protein whose precise function is not yet known. Recent studies propose divergent functions for CLN3; as a vesicular trafficking hub or its role in glycerophosphodiester (GPD) efflux from lysosomes. The role of CLN3 in the neurons and at the synapse remains elusive. Mutations in CLN3 gene cause Juvenile Neuronal Ceroid Lipofuscinosis (JNCL), a condition associated with learning impairments and memory deficits in affected children. Using CLN3Δex7/8 mouse model, we investigated the impact of CLN3 loss at synapses. Analysis of synaptic transmission revealed a signific

The lysosomal membrane protein, TMEM106b, has been identified as a risk factor for frontotemporal lobar degeneration with TDP-43 inclusions (FTLD-TDP). Although the cellular function of TMEM106b is poorly understood, it has been shown to regulate lysosomal morphology and trafficking and autophagy in neurons. Recently, there has been interest in the discovery of TMEM106b amyloid fibrils formed by its lumenal C-terminus, which have been observed in various neurodegenerative disorders and healthy aged brain. In a high-throughput phosphoproteomic screen, we identified TMEM106b as a substrate for t