Results 21 to 30 of about 29,691,692 (162)

Lung emphysema and impaired macrophage elastase clearance in mucolipin 3 deficient mice [PDF]

, 2022
peer reviewedLung emphysema and chronic bronchitis are the two most common causes of chronic obstructive pulmonary disease. Excess macrophage elastase MMP-12, which is predominantly secreted from alveolar macrophages, is known to mediate the development ...
Biel, Martin, Tang, R., Wyatt, Amanda, Ansari, M., Wartenberg, Philipp, Boehm, Ulrich, Zierler, Susanna, Holdt, Lesca M., Giera, M., Biel, M., Saftig, P., Schiller, H.B., Spix, B., Conlon, T.M., Butz, E.S., Schiller, Herbert B., Kobold, S., Gudermann, T., Yildirim, Ali, Boehm, U., Schiller, H. B., Butz, Elisabeth S., Holdt, Lesca, Kobold, Sebastian, Günsel, Gizem Günes, Wartenberg, P., Zierler, S., Rosato, Anna Scotto, Wyatt, A., Saftig, Paul, Bracher, F., Jeridi, A., Ectors, Fabien, Conlon, Thomas M., Briukhovetska, D., Teupser, D., Holdt, L.M., Ansari, Meshal, Günes, Gizem, Scotto Rosato, Anna, Arlt, Elisabeth, Briukhovetska, Daria, Bracher, Franz, Boekhoff, I., Wahl-Schott, C., Ectors, F., Garcia-Anoveros, Jaime, Giera, Martin, Yildirim, A.Ö., Schiller, Herbert, Wahl-Schott, Christian, Grimm, C., Jeridi, Aicha, Wetzel, S., Günsel, G.G., Tang, Rachel, Arlt, E., Conlon, Thomas, Jeridi,, Grimm, Christian, Rosato, A.S., García-Añoveros, J., Spix, Barbara, Butz, Elisabeth, Wetzel, Sandra, Kudrina, V., Chen, Cheng-Chang, Boekhoff, Ingrid, Yildirim, Ali Önder, Kudrina, Veronika, Gudermann, Thomas, Chen, C.C., Plesch, Eva, Teupser, Daniel, Plesch, E.   +74 more
core   +1 more source

A novel ion channel formed by interaction of TRPML3 with TRPV5. [PDF]

, 2013
TRPML3 and TRPV5 are members of the mucolipin (TRPML) and TRPV subfamilies of transient receptor potential (TRP) cation channels. Based on sequence similarities of the pore forming regions and on structure-function evidence, we hypothesized that the pore
Becker, Lars, Anthony J Ricci, Grimm, Christian, Heller, Stefan, Anthony J. Ricci (212303), Ricci, Anthony J., Stefan Heller, Lars Becker (358879), Zhaohua Guo (383295), Zhaohua Guo, Stefan Heller (36641), Guo, Zhaohua, Lars Becker, Christian Grimm, Christian Grimm (84987)   +14 more
core   +2 more sources

Molecular pathophysiology of mucolipidosis type IV: pH dysregulation of the mucolipin-1 cation channel [PDF]

Human Molecular Genetics, 2004
Mucolipidosis type IV (MLIV) is an autosomal recessive neurogenetic disorder characterized by developmental abnormalities of the brain and impaired neurological, ophthalmologic and gastric function. Large vacuoles accumulate in various types of cells in MLIV patients.
Malay K, Raychowdhury, Silvia, González-Perrett, Nicolás, Montalbetti, Gustavo A, Timpanaro, Bernard, Chasan, Wolfgang H, Goldmann, Stefanie, Stahl, Adele, Cooney, Ehud, Goldin, Horacio F, Cantiello   +9 more
openaire   +2 more sources

Pathophysiological Role of Transient Receptor Potential Mucolipin Channel 1 in Calcium-Mediated Stress-Induced Neurodegenerative Diseases

Frontiers in Physiology, 2020
Mucolipins (TRPML) are endosome/lysosome Ca2+ permeable channels belonging to the family of transient receptor potential channels. In mammals, there are three TRPML proteins, TRPML1, 2, and 3, encoded by MCOLN1-3 genes.
Giorgio Santoni, Federica Maggi, Federica Maggi, Consuelo Amantini, Oliviero Marinelli, Oliviero Marinelli, Massimo Nabissi, Maria Beatrice Morelli, Maria Beatrice Morelli   +8 more
doaj   +1 more source

Lysosomal Zn2+ release triggers rapid, mitochondria-mediated, non-apoptotic cell death in metastatic melanoma

Cell Reports, 2021
Summary: During tumor progression, lysosome function is often maladaptively upregulated to match the high energy demand required for cancer cell hyper-proliferation and invasion. Here, we report that mucolipin TRP channel 1 (TRPML1), a lysosomal Ca2+ and
Wanlu Du, Mingxue Gu, Meiqin Hu, Prateeksunder Pinchi, Wei Chen, Michael Ryan, Timothy Nold, Ahmed Bannaga, Haoxing Xu   +8 more
doaj   +1 more source

N-butyldeoxynojirimycin delays motor deficits, cerebellar microgliosis, and Purkinje cell loss in a mouse model of mucolipidosis type IV

Neurobiology of Disease, 2017
Mucolipidosis type IV (MLIV) is a lysosomal storage disease exhibiting progressive intellectual disability, motor impairment, and premature death. There is currently no cure or corrective treatment. The disease results from mutations in the gene encoding
Lauren C. Boudewyn, Jakub Sikora, Ladislav Kuchar, Jana Ledvinova, Yulia Grishchuk, Shirley L. Wang, Kostantin Dobrenis, Steven U. Walkley   +7 more
doaj   +1 more source

The mucolipin-1 TRPML1 ion channel transmembrane-163 TMEM163 protein and lysosomal zinc handling [PDF]

Frontiers in Bioscience, 2017
Lysosomes are emerging as important players in cellular zinc ion (Zn2+) homeostasis. The series of work on Zn2+ accumulation in the neuronal lysosomes and the mounting evidence on the role of lysosomal Zn2+ in cell death during mammary gland involution set a biological precedent for the central role of the lysosomes in cellular Zn2+ handling.
Math P, Cuajungco, Kirill, Kiselyov
openaire   +2 more sources

ML-SA1, a TRPML1 agonist, induces gastric secretion and gastrointestinal tract inflammation in vivo

STEMedicine, 2020
Background: The effect of ML-SA1, a potent and specific TRPML1 channel agonist, on gastric secretion and subsequent impact to the gastrointestinal (GI) tract of mice was investigated. Methods: Twenty mice were divided into two groups, and respectively
Dan Gong, Jing Hai, Jun Ma, Chen-Xi Wang, Xin-Dan Zhang, Ya-Nan Xiang, Tao Tan, Ya-Nan Liu, Wei Zhang   +8 more
doaj   +1 more source

Functional multimerization of mucolipin channel proteins [PDF]

, 2010
MCOLN1 encodes mucolipin-1 (TRPML1), a member of the transient receptor potential TRPML subfamily of channel proteins. Mutations in MCOLN1 cause mucolipidosis-type IV (MLIV), a lysosomal storage disorder characterized by severe neurologic, ophthalmologic,
Zhang, Peng, Curcio Morelli, Cyntia, Cantiello, Horacio Fabio, Browning, Marsha F., Venugopal, Bhuvarahamurthy, Slaugenhaupt, Susan A., Charles, Florie A.   +6 more
core   +1 more source

Mucolipin 1 positively regulates TLR7 responses in dendritic cells by facilitating RNA transportation to lysosomes [PDF]

International Immunology, 2014
Abstract Toll-like receptor 7 (TLR7) and TLR9 sense microbial single-stranded RNA (ssRNA) and ssDNA in endolysosomes. Nucleic acid (NA)-sensing in endolysosomes is thought to be important for avoiding TLR7/9 responses to self-derived NAs. Aberrant self-derived NA transportation to endolysosomes predisposes to autoimmune diseases.
Xiaobing, Li, Shin-Ichiroh, Saitoh, Takuma, Shibata, Natsuko, Tanimura, Ryutaro, Fukui, Kensuke, Miyake   +5 more
openaire   +2 more sources