Results 31 to 40 of about 3,697 (189)

TRPML1 in Autophagy and Neuromuscular Diseases [PDF]

open access: yes, 2023
In this thesis, we used two neuromuscular disease models, i.e., XLMTM and ALS, to study the effects of TRPML1 on autophagy and its relevance to disease progression and treatment.
Syeda, Alia Kazim R.
core   +2 more sources

Knock-Down of Mucolipin 1 Channel Promotes Tumor Progression and Invasion in Human Glioblastoma Cell Lines

open access: yesFrontiers in Oncology, 2021
Among cancers that affect the central nervous system, glioblastoma is the most common. Given the negative prognostic significance of transient receptor potential mucolipin 1 (TRPML1) channel reduction in patients with glioblastoma, as discussed in ...
Giorgio Santoni   +8 more
doaj   +1 more source

TRPML1 as lysosomal fusion guard [PDF]

open access: yesChannels, 2016
restriction
Malini, Ahuja   +3 more
openaire   +3 more sources

Igniting Ca 2+ sparks with TRPML1 [PDF]

open access: yesProceedings of the National Academy of Sciences, 2020
Storage and voiding of urine in mammals is accomplished by a reciprocal contractile relationship between the bladder and the urethra. During the storage phase, the urethra remains contracted to prevent leakage of urine, while the bladder is relaxed to accommodate the increased volume of urine.
Gerard P, Sergeant   +2 more
openaire   +2 more sources

Mucolipins: Intracellular TRPML1‐3 channels [PDF]

open access: yesFEBS Letters, 2010
The mucolipin family of Transient Receptor Potential (TRPML) proteins is predicted to encode ion channels expressed in intracellular endosomes and lysosomes. Loss‐of‐function mutations of human TRPML1 cause type IV mucolipidosis (ML4), a childhood neurodegenerative disease.
Cheng, Xiping   +3 more
openaire   +3 more sources

Structure of mammalian endolysosomal TRPML1 channel in nanodiscs [PDF]

open access: yesNature, 2017
Transient receptor potential mucolipin 1 (TRPML1) is a cation channel located within endosomal and lysosomal membranes. Ubiquitously expressed in mammalian cells, its loss-of-function mutations are the direct cause of type IV mucolipidosis, an autosomal recessive lysosomal storage disease.
Chen, Qingfeng   +6 more
openaire   +2 more sources

Survival of intracellular pathogens in response to mTORC1- or TRPML1-TFEB-induced xenophagy

open access: yesAutophagy Reports, 2023
Intracellular pathogens establish persistent infections by generating reservoirs that protect them from the action of antibiotics and the host immune response.
Mariana I. Capurro   +3 more
doaj   +1 more source

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

open access: yesFrontiers 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   +13 more
doaj   +1 more source

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

open access: yesDisease 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   +9 more
doaj   +1 more source

The synthetic TRPML1 agonist ML-SA1 rescues Alzheimer-related alterations of the endosomal-autophagic-lysosomal system [PDF]

open access: yes, 2023
Abnormalities in the endosomal-autophagic-lysosomal (EAL) system are an early event in Alzheimer's disease (AD) pathogenesis. However, the mechanisms underlying these abnormalities are unclear. The transient receptor potential channel mucolipin 1(TRPML1,
Winston, Jincy   +12 more
core   +1 more source

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