TMEM106B

TMEM106B (Transmembrane Protein 106B) is a Protein Coding gene. Diseases associated with TMEM106B include Frontotemporal Dementia and Progressive Non-Fluent Aphasia. An important paralog of this gene is TMEM106C.

TMEM106B Transmembrane Protein 106B

In neurons, involved in the transport of late endosomes/lysosomes (PubMed:25066864).
May be involved in dendrite morphogenesis and maintenance by regulating lysosomal trafficking (PubMed:25066864).
May act as a molecular brake for retrograde transport of late endosomes/lysosomes, possibly via its interaction with MAP6 (By similarity).
In motoneurons, may mediate the axonal transport of lysosomes and axonal sorting at the initial segment (By similarity).
It remains unclear whether TMEM106B affects the transport of moving lysosomes in the anterograde or retrograde direction in neurites and whether it is important in the sorting of lysosomes in axons or in dendrites (By similarity).
In neurons, may also play a role in the regulation of lysosomal size and responsiveness to stress (PubMed:25066864).
Required for proper lysosomal acidification (By similarity).
(Microbial infection) May act as a proviral host factor for SARS-CoV-2, but not for common cold coronaviruses HCoV-229E and HCoV-OC43.

Biological Process dendrite morphogenesisSource:UniProtKB1 Publication
Biological Process lysosomal lumen acidificationSource:Ensembl
Biological Process lysosomal protein catabolic processSource:Ensembl
Biological Process lysosomal transportSource:GO_Central1 Publication
Biological Process lysosome localizationSource:UniProtKB1 Publication
Biological Process lysosome organizationSource:GO_Central1 Publication
Biological Process neuron cellular homeostasisSource:Ensembl
Biological Process positive regulation of dendrite developmentSource:Ensembl
Biological Process positive regulation of hydrolase activitySource:Ensembl
Biological Process regulation of lysosome organizationSource:Ensembl

Late endosome membrane
Lysosome membrane
Colocalizes with LAMP1.

Ubiquitin-positive frontotemporal dementia (UP-FTD):
Frontotemporal dementia (FTD) is the second most common cause of dementia in people under the age of 65 years. It is an autosomal dominant neurodegenerative disease.
Frontotemporal dementia and/or amyotrophic lateral sclerosis 1 (FTDALS1):
An autosomal dominant neurodegenerative disorder characterized by adult onset of frontotemporal dementia and/or amyotrophic lateral sclerosis in an affected individual. There is high intrafamilial variation. Frontotemporal dementia is characterized by frontal and temporal lobe atrophy associated with neuronal loss, gliosis, and dementia. Patients exhibit progressive changes in social, behavioral, and/or language function. Amyotrophic lateral sclerosis is characterized by the death of motor neurons in the brain, brainstem, and spinal cord, resulting in fatal paralysis.
Leukodystrophy, hypomyelinating, 16 (HLD16):
An autosomal dominant disorder characterized by hypomyelination, leukodystrophy, and thin corpus callosum observed on brain imaging. Clinical features include hypotonia, nystagmus, and mildly delayed motor development with onset in infancy, ataxic or broad-based gait, hyperreflexia, intention tremor, dysmetria, and a mild pyramidal syndrome. Some patients have cognitive impairment, whereas others may have normal cognition or mild intellectual disability with speech difficulties.

Cytoplasmic: 2-96
Helical: 97-117
Lumenal: 118-274