Results 41 to 50 of about 32,016,499 (171)
Palmitoyl-protein thioesterase-1 deficiency leads to the activation of caspase-9 and contributes to rapid neurodegeneration in INCL [PDF]
Human Molecular Genetics, 2006 The infantile neuronal ceroid lipofuscinosis (INCL), a rare (one in 100 000 births) but one of the most lethal inherited neurodegenerative storage disorders of childhood, is caused by inactivating mutations in the palmitoyl-protein thioesterase-1 (PPT1) gene.
Sung-Jo, Kim +3 more
openaire +2 more sources
Lysosomal lipid peroxidation mediates immunogenic cell death
The Journal of Clinical Investigation, 2023 Cancer cells rely on lysosome-dependent degradation to recycle nutrients that serve their energetic and biosynthetic needs. Despite great interest in repurposing the antimalarial hydroxychloroquine as a lysosomal inhibitor in clinical oncology trials ...
Pravin Phadatare, Jayanta Debnath
doaj +1 more source
Palmitoyl-protein thioesterase-1 deficiency mediates the activation of the unfolded protein response and neuronal apoptosis in INCL [PDF]
Human Molecular Genetics, 2005 Numerous proteins undergo modification by palmitic acid (S-acylation) for their biological functions including signal transduction, vesicular transport and maintenance of cellular architecture. Although palmitoylation is an essential modification, these proteins must also undergo depalmitoylation for their degradation by lysosomal proteases.
Zhongjian, Zhang +9 more
openaire +2 more sources
Treatment of CLN1 disease with a blood-brain barrier penetrating lysosomal enzyme
Molecular Genetics and Metabolism Reports, 2022 Neuronal ceroid lipofuscinosis type 1(CLN1 disease) is a rare autosomal recessive lysosomal storage disease caused by genetic defects of palmitoyl protein thioesterase-1(PPT1), leading to accumulation of lipofuscin granules in brain and progressive ...
Andreas Hahn +7 more
doaj +1 more source
, 2011 Copyright information:Taken from "Glycosylation, transport, and complex formation of palmitoyl protein thioesterase 1 (PPT1) – distinct characteristics in neurons"http://www.biomedcentral.com/1471-2121/8/22BMC Cell Biology 2007;8():22-22.Published online
Carina von Schantz (45483) +7 more
core +1 more source
The role of palmitoyl–protein thioesterase in the palmitoylation of endothelial nitric oxide synthase [PDF]
, 1997 Palmitoylation of eNOS is required for targeting to plasmalemmal caveolae and agonist-promoted depalmitoylation leads to eNOS translocation, modifying the agonist response.
Michel, Jeffrey B. +3 more
core +1 more source
, 2011 Copyright information:Taken from "Glycosylation, transport, and complex formation of palmitoyl protein thioesterase 1 (PPT1) – distinct characteristics in neurons"http://www.biomedcentral.com/1471-2121/8/22BMC Cell Biology 2007;8():22-22.Published online
Carina von Schantz (45483) +7 more
core +1 more source
, 2011 Copyright information:Taken from "Glycosylation, transport, and complex formation of palmitoyl protein thioesterase 1 (PPT1) – distinct characteristics in neurons"http://www.biomedcentral.com/1471-2121/8/22BMC Cell Biology 2007;8():22-22.Published online
Carina von Schantz (45483) +7 more
core +1 more source
eLife, 2019 Protein palmitoylation and depalmitoylation alter protein function. This post-translational modification is critical for synaptic transmission and plasticity.
Kevin P Koster +5 more
doaj +1 more source
, 2011 Copyright information:Taken from "Glycosylation, transport, and complex formation of palmitoyl protein thioesterase 1 (PPT1) – distinct characteristics in neurons"http://www.biomedcentral.com/1471-2121/8/22BMC Cell Biology 2007;8():22-22.Published online
Carina von Schantz (45483) +7 more
core +1 more source