Results 151 to 160 of about 3,697 (189)

Reviewer #1 (Public Review): TRPML1 gating modulation by allosteric mutations and lipids (Design of allosteric mutations that recapitulate the gating of TRPML1)

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Ca ²⁺ and DRP1 drive endocytic lysosome reformation at tripartite contact sites

Desai S, Martín-Sánchez E, Ritz D, Schmidt A, Spang A.   +4 more
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Multiple facets of TRPML1 in autophagy

Cell Calcium, 2020
Autophagy is an evolutionarily conserved pathway that is required for cellular homeostasis, growth and survival. In a recent study, Scotto-Rosato et al. demonstrate that TRPML1-mediated calcium release promotes autophagosome biogenesis by activating the CaMKKβ/VPS34 pathway, providing a new insight into the pathophysiological role of TRPML1 in human ...
Xian-Ping Dong, Mengnan Xu
exaly   +3 more sources

Increased expression or activation of TRPML1 reduces hepatic storage of toxic Z alpha-1 antitrypsin

Molecular Therapy, 2023
: Mutant Z-alpha-1 antitrypsin (ATZ) accumulates in globules in the liver and is the prototype of proteotoxic hepatic disease. Therapeutic strategies aiming at clearance of polymeric ATZ are needed.
Edoardo Nusco
exaly   +2 more sources

HRAS‐driven cancer cells are vulnerable to TRPML1 inhibition [PDF]

EMBO Reports, 2019
By serving as intermediaries between cellular metabolism and the bioenergetic demands of proliferation, endolysosomes allow cancer cells to thrive under normally detrimental conditions. Here, we show that an endolysosomal TRP channel, TRPML1, is necessary for the proliferation of cancer cells that bear activating mutations in HRAS Expression of MCOLN1,
Yong Zhou, Ilya Levental, Kwang-Jin Cho   +2 more
exaly   +3 more sources

Atomic insights into ML-SI3 mediated human TRPML1 inhibition

Structure, 2021
Transient receptor potential mucolipin 1 (TRPML1) regulates lysosomal calcium signaling, lipid trafficking, and autophagy-related processes. This channel is regulated by phosphoinositides and the low pH environment of the lysosome, maintaining calcium levels essential for proper lysosomal function.
Philip Schmiege, Michael Fine, Xiaochun Li   +2 more
exaly   +3 more sources

Mutation of TRPML1 Channel and Pathogenesis of Neurodegeneration in Haimeria

Molecular Neurobiology, 2023
Neurodegenerative diseases, a group of debilitating disorders, have garnered increasing attention due to their escalating prevalence, particularly among aging populations. Alzheimer's disease (AD) reigns as a prominent exemplar within this category, distinguished by its relentless progression of cognitive impairment and the accumulation of aberrant ...
Junqing Gao, Huanhuan Li, Hua Lv, Xiansong Cheng   +3 more
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A Structural Overview of TRPML1 and the TRPML Family

2022
This chapter explores the existing structural and functional studies on the endo-lysosomal channel TRPML1 and its analogs TRPML2, TRPML3. These channels represent the mucolipin subfamily of the TRP channel superfamily comprising important roles in sensory physiology, ion homeostasis, and signal transduction.
Michael, Fine, Xiaochun, Li
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TRPML1

2010
TRPML1 (or mucolipin 1) is the first member of the TRP family of ion channels that was found to function in the lower portions of the endocytic pathway. Mutations in the gene coding for TRPML1 (MCOLN1) cause the lysosomal storage disease mucolipidosis type IV (MLIV).
Grace A, Colletti, Kirill, Kiselyov
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