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Pedram Argani et al.
The American journal of surgical pathology, 27(6), 750-761 (2003-05-27)
We report the aberrantly strong nuclear immunoreactivity for the C-terminal portion of TFE3 protein in tumors characterized by chromosome translocations involving the TFE3 gene at Xp11.2. This group of tumors includes alveolar soft part sarcoma and a specific subset of
Identification of Transcription Factor E3 (TFE3) as a Receptor-independent Activator of G alpha 16 GENE REGULATION BY NUCLEAR G alpha SUBUNIT AND ITS ACTIVATOR
Sato M, et al.
The Journal of Biological Chemistry, 286(20), 17766-17776 (2011)
Rosa Bartolomeo et al.
The Journal of clinical investigation, 127(10), 3717-3729 (2017-09-06)
The mammalian target of rapamycin complex 1 (mTORC1) kinase promotes cell growth by activating biosynthetic pathways and suppressing catabolic pathways, particularly that of macroautophagy. A prerequisite for mTORC1 activation is its translocation to the lysosomal surface. Deregulation of mTORC1 has
J Mathieu et al.
Nature communications, 10(1), 632-632 (2019-02-09)
To reveal how cells exit human pluripotency, we designed a CRISPR-Cas9 screen exploiting the metabolic and epigenetic differences between naïve and primed pluripotent cells. We identify the tumor suppressor, Folliculin(FLCN) as a critical gene required for the exit from human
Shaogui Wang et al.
Autophagy, 15(11), 1954-1969 (2019-03-22)
Impaired macroautophagy/autophagy has been implicated in experimental and human pancreatitis. However, the transcriptional control governing the autophagy-lysosomal process in pancreatitis is largely unknown. We investigated the role and mechanisms of TFEB (transcription factor EB), a master regulator of lysosomal biogenesis
ERK-independent African Green monkey pluripotent stem cells in a putative chimera-competent state
De Los Angeles A, et al.
Biochemical and Biophysical Research Communications, 510(1), 78-84 (2019)
Nunzia Pastore et al.
Autophagy, 12(8), 1240-1258 (2016-05-14)
The activation of transcription factors is critical to ensure an effective defense against pathogens. In this study we identify a critical and complementary role of the transcription factors TFEB and TFE3 in innate immune response. By using a combination of
Joerg Betschinger et al.
Cell, 153(2), 335-347 (2013-04-16)
Factors that sustain self-renewal of mouse embryonic stem cells (ESCs) are well described. In contrast, the machinery regulating exit from pluripotency is ill defined. In a large-scale small interfering RNA (siRNA) screen, we found that knockdown of the tumor suppressors
Shuyan Lu et al.
PloS one, 12(3), e0173771-e0173771 (2017-03-17)
Lysosomes are acidic organelles essential for degradation and cellular homoeostasis and recently lysosomes have been shown as signaling hub to respond to the intra and extracellular changes (e.g. amino acid availability). Compounds including pharmaceutical drugs that are basic and lipophilic
Ismail Sergin et al.
Nature communications, 8, 15750-15750 (2017-06-08)
Macrophages specialize in removing lipids and debris present in the atherosclerotic plaque. However, plaque progression renders macrophages unable to degrade exogenous atherogenic material and endogenous cargo including dysfunctional proteins and organelles. Here we show that a decline in the autophagy-lysosome
Leeanna El-Houjeiri et al.
Cell reports, 26(13), 3613-3628 (2019-03-28)
TFEB and TFE3 are transcriptional regulators of the innate immune response, but the mechanisms regulating their activation upon pathogen infection are poorly elucidated. Using C. elegans and mammalian models, we report that the master metabolic modulator 5'-AMP-activated protein kinase (AMPK) and
Shogo Wada et al.
Genes & development, 30(22), 2551-2564 (2016-12-04)
Noncanonical mechanistic target of rapamycin (mTOR) pathways remain poorly understood. Mutations in the tumor suppressor folliculin (FLCN) cause Birt-Hogg-Dubé syndrome, a hamartomatous disease marked by mitochondria-rich kidney tumors. FLCN functionally interacts with mTOR and is expressed in most tissues, but
Xiaoli Zhang et al.
Nature communications, 7, 12109-12109 (2016-07-01)
Cellular stresses trigger autophagy to remove damaged macromolecules and organelles. Lysosomes 'host' multiple stress-sensing mechanisms that trigger the coordinated biogenesis of autophagosomes and lysosomes. For example, transcription factor (TF)EB, which regulates autophagy and lysosome biogenesis, is activated following the inhibition
Nicole Merritt et al.
eLife, 10 (2021-04-30)
Epithelioid hemangioendothelioma (EHE) is a vascular sarcoma that metastasizes early in its clinical course and lacks an effective medical therapy. The TAZ-CAMTA1 and YAP-TFE3 fusion proteins are chimeric transcription factors and initiating oncogenic drivers of EHE. A combined proteomic/genetic screen
Maria De Luca et al.
Scientific reports, 11(1), 4615-4615 (2021-02-27)
V-ATPase is a large multi-subunit complex that regulates acidity of intracellular compartments and of extracellular environment. V-ATPase consists of several subunits that drive specific regulatory mechanisms. The V1G1 subunit, a component of the peripheral stalk of the pump, controls localization
TFE3 regulates renal adenocarcinoma cell proliferation via activation of the mTOR pathway
Fang Y, et al.
Molecular Medicine Reports, 16(3), 2721-2725 (2017)
Seung-Beom Hong et al.
PloS one, 5(12), e15793-e15793 (2011-01-07)
Germline mutations in a tumor suppressor gene FLCN lead to development of fibrofolliculomas, lung cysts and renal cell carcinoma (RCC) in Birt-Hogg-Dubé syndrome. TFE3 is a member of the MiTF/TFE transcription factor family and Xp11.2 translocations found in sporadic RCC
Ohad Gafni et al.
Nature, 504(7479), 282-286 (2013-11-01)
Mouse embryonic stem (ES) cells are isolated from the inner cell mass of blastocysts, and can be preserved in vitro in a naive inner-cell-mass-like configuration by providing exogenous stimulation with leukaemia inhibitory factor (LIF) and small molecule inhibition of ERK1/ERK2
Epithelioid hemangioendotheliomas with TFE3 gene translocations are compossible with CAMTA1 gene rearrangements
Lee SJ, et al.
Testing, 7(7), 7480-7480 (2016)
Matteo Bordi et al.
Autophagy, 12(12), 2467-2483 (2016-11-05)
Defective autophagy contributes to Alzheimer disease (AD) pathogenesis although evidence is conflicting on whether multiple stages are impaired. Here, for the first time, we have comprehensively evaluated the entire autophagic process specifically in CA1 pyramidal neurons of hippocampus from early
Ida Annunziata et al.
Nature communications, 10(1), 3623-3623 (2019-08-11)
Coordinated regulation of the lysosomal and autophagic systems ensures basal catabolism and normal cell physiology, and failure of either system causes disease. Here we describe an epigenetic rheostat orchestrated by c-MYC and histone deacetylases that inhibits lysosomal and autophagic biogenesis
Huifeng Pi et al.
Autophagy, 15(4), 565-582 (2018-10-17)
Cadmium (Cd) is a toxic metal that is widely found in numerous environmental matrices and induces serious adverse effects in various organs and tissues. Bone tissue seems to be a crucial target of Cd contamination. Macroautophagy/autophagy has been proposed to
Dun-Sheng Yang et al.
Human molecular genetics, 26(5), 843-859 (2017-01-08)
2-hydroxypropyl-β-cyclodextrin (CYCLO), a modifier of cholesterol efflux from cellular membrane and endo-lysosomal compartments, reduces lysosomal lipid accumulations and has therapeutic effects in animal models of Niemann-Pick disease type C and several other neurodegenerative states. Here, we investigated CYCLO effects on
José A Martina et al.
Science signaling, 7(309), ra9-ra9 (2014-01-23)
The discovery of a gene network regulating lysosomal biogenesis and its transcriptional regulator transcription factor EB (TFEB) revealed that cells monitor lysosomal function and respond to degradation requirements and environmental cues. We report the identification of transcription factor E3 (TFE3)
Huan Du et al.
Life science alliance, 4(7) (2021-06-10)
Haploinsufficiency of progranulin (PGRN) is a leading cause of frontotemporal lobar degeneration (FTLD). PGRN polymorphisms are associated with Alzheimer's disease. PGRN is highly expressed in the microglia near Aβ plaques and influences plaque dynamics and microglial activation. However, the detailed
Yueyang Liu et al.
Autophagy, 15(3), 493-509 (2018-10-12)
Mounting attention has been focused on defects in macroautophagy/autophagy and the autophagy-lysosomal pathway (ALP) in cerebral ischemia. TFEB (transcription factor EB)-mediated induction of ALP has been recently considered as the common mechanism in ameliorating the pathological lesion of myocardial ischemia
Folliculin Regulates Osteoclastogenesis Through Metabolic Regulation
Baba M, et al.
Journal of Bone and Mineral Research, 33(10), 1785-1798 (2018)
Daniela Cornacchia et al.
Cell stem cell, 25(1), 120-136 (2019-06-04)
Current challenges in capturing naive human pluripotent stem cells (hPSCs) suggest that the factors regulating human naive versus primed pluripotency remain incompletely defined. Here we demonstrate that the widely used Essential 8 minimal medium (E8) captures hPSCs at a naive-to-primed intermediate
Ruslana Bryk et al.
PLoS pathogens, 16(6), e1008567-e1008567 (2020-06-24)
Efforts at host-directed therapy of tuberculosis have produced little control of the disease in experimental animals to date. This is not surprising, given that few specific host targets have been validated, and reciprocally, many of the compounds tested potentially impact
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