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J Cell Sci. 2016 Jul 1;129(13):2475-81. doi: 10.1242/jcs.146365. Epub 2016 Jun 1.

TFEB at a glance.

Author information

1
Telethon Institute of Genetics and Medicine (TIGEM), 80131 Naples, Italy.
2
Telethon Institute of Genetics and Medicine (TIGEM), 80131 Naples, Italy Medical Genetics, Department of Translational Medicine, Federico II University, 80131 Naples, Italy Department of Molecular and Human Genetics, Baylor College of Medicine, Houston, TX 77030, USA Jan and Dan Duncan Neurological Research Institute, Texas Children Hospital, Houston, TX 77030, USA ballabio@tigem.it.

Abstract

The transcription factor EB (TFEB) plays a pivotal role in the regulation of basic cellular processes, such as lysosomal biogenesis and autophagy. The subcellular localization and activity of TFEB are regulated by mechanistic target of rapamycin (mTOR)-mediated phosphorylation, which occurs at the lysosomal surface. Phosphorylated TFEB is retained in the cytoplasm, whereas dephosphorylated TFEB translocates to the nucleus to induce the transcription of target genes. Thus, a lysosome-to-nucleus signaling pathway regulates cellular energy metabolism through TFEB. Recently, in vivo studies have revealed that TFEB is also involved in physiological processes, such as lipid catabolism. TFEB has attracted a lot of attention owing to its ability to induce the intracellular clearance of pathogenic factors in a variety of murine models of disease, such as Parkinson's and Alzheimer's, suggesting that novel therapeutic strategies could be based on the modulation of TFEB activity. In this Cell Science at a Glance article and accompanying poster, we present an overview of the latest research on TFEB function and its implication in human diseases.

KEYWORDS:

Autophagy; Lysosomal storage disorders; Lysosomes; MiT family; TFE3; TFEB; mTOR

PMID:
27252382
PMCID:
PMC4958300
DOI:
10.1242/jcs.146365
[Indexed for MEDLINE]
Free PMC Article

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