DataSheet1_The Dictyostelium Model for Mucolipidosis Type IV.PDF
, 2022 Mucolipidosis type IV, a devastating neurological lysosomal disease linked to mutations in the transient receptor potential channel mucolipin 1, TRPML1, a calcium permeable channel in the membranes of vesicles in endolysosomal system.
Claire Y. Allan (12409690) +1 more
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Impaired myelination and reduced brain ferric iron in the mouse model of mucolipidosis IV
Disease Models & Mechanisms, 2015 Mucolipidosis type IV (MLIV) is a lysosomal storage disease caused by mutations in the MCOLN1 gene, which encodes the lysosomal transient receptor potential ion channel mucolipin-1 (TRPML1).
Yulia Grishchuk +7 more
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The MADD-3 LAMMER Kinase Interacts with a p38 MAP Kinase Pathway to Regulate the Display of the EVA-1 Guidance Receptor in Caenorhabditis elegans. [PDF]
PLoS Genetics, 2016 The proper display of transmembrane receptors on the leading edge of migrating cells and cell extensions is essential for their response to guidance cues.
Serena A D'Souza +6 more
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Ion Channels and Pumps in Autophagy: A Reciprocal Relationship
Cells, 2021 Autophagy, the process of cellular self-degradation, is intrinsically tied to the degradative function of the lysosome. Several diseases have been linked to lysosomal degradative defects, including rare lysosomal storage disorders and neurodegenerative ...
Hussein Abuammar +6 more
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The activation of Mucolipin TRP channel 1 (TRPML1) protects motor neurons from L-BMAA neurotoxicity by promoting autophagic clearance [PDF]
, 2019 Cellular clearance mechanisms including the autophagy-lysosome pathway are impaired in amyotrophic lateral sclerosis (ALS). One of the most important proteins involved in the regulation of autophagy is the lysosomal Ca2+ channel Mucolipin TRP channel 1 ...
Petrozziello, Tiziana +5 more
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Antioxidants, 2022 Doxorubicin (DOX), a widely used chemotherapeutic agent, has been linked to an increased risk of bone damage in human patients and induces bone loss in mice. DOX induces autophagy, which contributes to bone homeostasis and excess autophagy in osteoclasts
Hyun-Jung Park +4 more
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Ecotoxicology and Environmental Safety, 2023 Aflatoxin B1 (AFB1) is a stable mycotoxin that contaminates animal feed on a large scale and causes severe damage to intestinal cells, induces inflammation and stimulates autophagy.
Xinyi Cheng +9 more
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Transient Receptor Potential Mucolipin 1 (TRPML1) and Two-pore Channels Are Functionally Independent Organellar Ion Channels [PDF]
Journal of Biological Chemistry, 2011 NAADP is a potent second messenger that mobilizes Ca(2+) from acidic organelles such as endosomes and lysosomes. The molecular basis for Ca(2+) release by NAADP, however, is uncertain. TRP mucolipins (TRPMLs) and two-pore channels (TPCs) are Ca(2+)-permeable ion channels present within the endolysosomal system.
Soichiro, Yamaguchi +8 more
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Lysosomal exocytosis and lipid storage disorders
Journal of Lipid Research, 2014 Lysosomes are acidic compartments in mammalian cells that are primarily responsible for the breakdown of endocytic and autophagic substrates such as membranes, proteins, and lipids into their basic building blocks. Lysosomal storage diseases (LSDs) are a
Mohammad Ali Samie, Haoxing Xu
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Inhibition of Transient Receptor Potential Channel Mucolipin-1 (TRPML1) by Lysosomal Adenosine Involved in Severe Combined Immunodeficiency Diseases [PDF]
Journal of Biological Chemistry, 2017 Impaired adenosine homeostasis has been associated with numerous human diseases. Lysosomes are referred to as the cellular recycling centers that generate adenosine by breaking down nucleic acids or ATP. Recent studies have suggested that lysosomal adenosine overload causes lysosome defects that phenocopy patients with mutations in transient receptor ...
Xi Zoë, Zhong +8 more
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