Results 21 to 30 of about 1,761,902 (161)

Next-Generation Sequencing Analysis Reveals Novel Pathogenic Variants in Four Chinese Siblings With Late-Infantile Neuronal Ceroid Lipofuscinosis

open access: yesFrontiers in Genetics, 2019
Neuronal Ceroid Lipofuscinoses (NCLs) are progressive degenerative diseases mainly affect brain and retina. They are characterized by accumulation of autofluorescent storage material, mitochondrial ATPase subunit C, or sphingolipid activator proteins A ...
Xiao-Tun Ren   +8 more
doaj   +1 more source

Sphingolipid-Transporting Proteins as Cancer Therapeutic Targets [PDF]

open access: yes, 2019
This article was supported by the German Research Foundation (DFG) and the Open Access Publication Fund of Humboldt-Universität zu Berlin.The understanding of the role of sphingolipid metabolism in cancer has tremendously increased in the past ten years.
Arenz, Christoph   +9 more
core   +1 more source

Disorders of Sphingolipid Synthesis, Sphingolipidoses, Niemann-Pick Disease Type C and Neuronal Ceroid Lipofuscinoses

open access: yes, 2022
International audienceSphingolipids are ubiquitous lipids found in all mammalian cell membranes and in plasma lipoproteins. Many exhibit dual functions, as key structural elements, but also as modulators of biological/physiological functions ...
Caillaud, Catherine   +2 more
core   +1 more source

The lysosomal trafficking of sphingolipid activator proteins (SAPs) is mediated by sortilin [PDF]

open access: yesThe EMBO Journal, 2003
Most soluble lysosomal proteins bind the mannose 6-phosphate receptor (M6P-R) to be sorted to the lysosomes. However, the lysosomes of I-cell disease (ICD) patients, a condition resulting from a mutation in the phosphotransferase that adds mannose 6-phosphate to hydrolases, have near normal levels of several lysosomal proteins, including the ...
Stephane, Lefrancois   +4 more
openaire   +2 more sources

Orm family proteins mediate sphingolipid homeostasis [PDF]

open access: yes, 2010
Despite the essential roles of sphingolipids both as structural components of membranes and critical signalling molecules, we have a limited understanding of how cells sense and regulate their levels.
Ruedi Aebersold   +28 more
core   +1 more source

Mechanisms of sphingolipid functions during heat stress in "Saccharomyces cerevisiae" [PDF]

open access: yes, 2005
Sphingolipids are important membrane constituents in all eukaryotic cells. Ongoing sphingolipid synthesis is required for a variety of cellular processes including response to heat shock.
Meier, Karsten D.
core   +1 more source

CPTP: A sphingolipid transfer protein that regulates autophagy and inflammasome activation [PDF]

open access: yesAutophagy, 2018
The macroautophagy/autophagy and inflammasome pathways are linked through their roles in innate immunity and chronic inflammatory disease. Ceramide-1-phosphate (C1P) is a bioactive sphingolipid that regulates pro-inflammatory eicosanoid production. Whether C1P also regulates autophagy and inflammasome assembly/activation is not known. Here we show that
Shrawan Kumar Mishra   +5 more
openaire   +3 more sources

Sphingolipid metabolism dysfunctions [PDF]

open access: yes, 2018
Sfingolipidi su glavne komponente staničnih membrana u živčanom sustavu svih eukariota. S obzirom da su uklopljeni u stanične membrane, oni kontroliraju i bitne stanične signalne puteve.
Hefer, Marija
core   +2 more sources

Do Sphingolipids Control Viral Membrane Protein Activity? [PDF]

open access: yesBiophysical Journal, 2011
The membrane-behaviour of the matrix (M) protein from respiratory syncytical virus, a prototypical enveloped virus, has been characterised by surface techniques (tensiometry, Brewster angle microscopy and atomic force microscopy) and experiments using lipid bilayers (FRET, light microscopy).
Sanderson, John M.   +3 more
openaire   +1 more source

List of putative FFAT-like domains found in proteins related to the sphingolipid synthesis.

open access: yes, 2018
List of putative FFAT-like domains found in proteins related to the sphingolipid synthesis.
Peter Mattjus (437084)   +5 more
core   +1 more source

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