Results 11 to 20 of about 29,691,692 (162)

Transient Receptor Potential Mucolipin-1 Channels in Glioblastoma: Role in Patient’s Survival [PDF]

Cancers, 2019
A link between mucolipin channels and tumors has been recently suggested. Herein, we aim to investigate the transient receptor potential mucolipin (TRPML)-1 relevance in glioblastoma. The expression of this channel was evaluated via qRT-PCR and immunohistochemistry in biopsies from 66 glioblastoma patients and two human glioblastoma cell lines and ...
Morelli, Maria Beatrice, Amantini, Consuelo, Tomassoni, Daniele, Nabissi, Massimo, Arcella, Antonella, Santoni, Giorgio   +5 more
openaire   +5 more sources

Rapamycin directly activates lysosomal mucolipin TRP channels independent of mTOR. [PDF]

PLoS Biology, 2019
Rapamycin (Rap) and its derivatives, called rapalogs, are being explored in clinical trials targeting cancer and neurodegeneration. The underlying mechanisms of Rap actions, however, are not well understood.
Xiaoli Zhang, Wei Chen, Qiong Gao, Junsheng Yang, Xueni Yan, Han Zhao, Lin Su, Meimei Yang, Chenlang Gao, Yao Yao, Ken Inoki, Dan Li, Rong Shao, Shiyi Wang, Nirakar Sahoo, Fumitaka Kudo, Tadashi Eguchi, Benfang Ruan, Haoxing Xu   +18 more
doaj   +3 more sources

LAPTMs regulate lysosomal function and interact with mucolipin 1: new clues for understanding mucolipidosis type IV [PDF]

Journal of Cell Science, 2011
Loss-of-function mutations in mucolipin 1 (MCOLN1) result in mucolipidosis type IV (MLIV), a lysosomal storage disorder characterized by severe mental and psychomotor retardation. MCOLN1 is a lysosomal ion channel that belongs to the transient receptor potential (TRP) superfamily.
Silvia, Vergarajauregui, Jose A, Martina, Rosa, Puertollano   +2 more
openaire   +3 more sources

Activated Endolysosomal Cation Channel TRPML1 Facilitates Maturation of α-Synuclein-Containing Autophagosomes

Frontiers in Cellular Neuroscience, 2022
Background: Protein aggregates are degraded via the autophagy-lysosome pathway and alterations in the lysosomal system leading to the accumulation of pathogenic proteins, including aggregates of α-synuclein in Parkinson’s disease (PD).
Maike R. Pollmanns, Judith Beer, Ines Rosignol, Ines Rosignol, Natalia Rodriguez-Muela, Natalia Rodriguez-Muela, Natalia Rodriguez-Muela, Björn H. Falkenburger, Björn H. Falkenburger, Björn H. Falkenburger, Björn H. Falkenburger, Elisabeth Dinter, Elisabeth Dinter, Elisabeth Dinter   +13 more
doaj   +1 more source

The inhibition of TRPML1/TFEB leads to lysosomal biogenesis disorder, contributes to developmental fluoride neurotoxicity

Ecotoxicology and Environmental Safety, 2023
Fluoride is capable of inducing developmental neurotoxicity; regrettably, the mechanism is obscure. We aimed to probe the role of lysosomal biogenesis disorder in developmental fluoride neurotoxicity—specifically, the regulating effect of the transient ...
Jingjing Zhang, Yanling Tang, Zeyu Hu, Wanjing Xu, Yue Ma, Panpan Xu, Hengrui Xing, Qiang Niu   +7 more
doaj   +1 more source

Ferritin Is Secreted from Primary Cultured Astrocyte in Response to Iron Treatment via TRPML1-Mediated Exocytosis

Cells, 2023
Impaired iron homeostasis has been proven to be one of the critical contributors to the pathology of Parkinson’s disease (PD). Ferritin is considered an intracellular protein responsible for storing cytosolic iron. Recent studies have found that ferritin
Xiaoqi Yu, Zhixin Xiao, Junxia Xie, Huamin Xu   +3 more
doaj   +1 more source

Posttranslational Cleavage and Adaptor Protein Complex-dependent Trafficking of Mucolipin-1 [PDF]

Journal of Biological Chemistry, 2006
Mucolipin-1 (ML1) is a member of the transient receptor potential ion channel superfamily that is thought to function in the biogenesis of lysosomes. Mutations in ML1 result in mucolipidosis type IV, a lysosomal storage disease characterized by the intracellular accumulation of enlarged vacuolar structures containing phospholipids, sphingolipids, and ...
Mark T, Miedel, Kelly M, Weixel, Jennifer R, Bruns, Linton M, Traub, Ora A, Weisz   +4 more
openaire   +2 more sources

Isolated Ocular Disease Is Associated with Decreased Mucolipin-1 Channel Conductance [PDF]

Investigative Opthalmology & Visual Science, 2008
To evaluate a 15-year-old boy with MLIV (mucolipidosis type IV) and clinical abnormalities restricted to the eye who also had achlorhydria with elevated blood gastrin levels.In addition to a detailed neuro-ophthalmic and electrophysiological assessment, his mutant mucolipin-1 was experimentally expressed in liposomes and its channel properties studied ...
Ehud, Goldin, Rafael C, Caruso, William, Benko, Christine R, Kaneski, Stephanie, Stahl, Raphael, Schiffmann   +5 more
openaire   +2 more sources

Early evidence of delayed oligodendrocyte maturation in the mouse model of mucolipidosis type IV

Disease Models & Mechanisms, 2020
Mucolipidosis type IV (MLIV) is a lysosomal disease caused by mutations in the MCOLN1 gene that encodes the endolysosomal transient receptor potential channel mucolipin-1, or TRPML1.
Molly Mepyans, Livia Andrzejczuk, Jahree Sosa, Sierra Smith, Shawn Herron, Samantha DeRosa, Susan A. Slaugenhaupt, Albert Misko, Yulia Grishchuk, Kirill Kiselyov   +9 more
doaj   +1 more source

TRPML1—Emerging Roles in Cancer

Cells, 2020
The mucolipin-1 (TRPML1) channel maintains lysosomal ionic homeostasis and regulates autophagic flux. Defects of TRPML1 lead to lysosomal storage diseases and neurodegeneration.
Yiming Yang, Xingjian Zhai, Yassine El Hiani   +2 more
doaj   +1 more source