Results 61 to 70 of about 6,648 (196)
Gaucher disease (GD) is a lysosomal storage disorder caused by mutations in GBA1, the gene that encodes lysosomal β‐glucocerebrosidase (GCase). Mild mutations in GBA1 cause type 1 non‐neuronopathic GD, whereas severe mutations cause types 2 and 3 ...
Manasa P. Srikanth +6 more
doaj +1 more source
Discovery and Evaluation of Inhibitors of Human Ceramidase [PDF]
Abstract The ceramide/sphingosine-1-phosphate (S1P) rheostat has been hypothesized to play a critical role in regulating tumor cell fate, with elevated levels of ceramide inducing death and elevated levels of S1P leading to survival and proliferation.
Draper, Jeremiah M +5 more
openaire +3 more sources
Polyploid giant cancer cells (PGCCs) arise in response to stress and play a critical role in therapy resistance and tumor dormancy. These cells can evade conventional treatments and later contribute to cancer relapse by generating progeny through nonmitotic, amitotic mechanisms.
Silvia Guil‐Luna +3 more
wiley +1 more source
Abstract Background Lysosomal dysfunction is central to Parkinson's disease (PD) pathogenesis, with GBA1 representing the strongest established genetic risk factor. Numerous other genes involved in lysosomal sphingolipid, glycosphingolipid, and ceramide metabolism have been proposed as contributors to PD, highlighting the need for genetic analyses ...
Konstantin Senkevich +21 more
wiley +1 more source
Summary: Ceramides and their metabolites are important for the homeostasis of the epidermis, but much remains unknown about the roles of specific pathways of ceramide metabolism in skin biology.
Chih-Li Lin +14 more
doaj +1 more source
Macrophages are critical in limiting replication of herpes simplex virus type 1 (HSV-1). Here the authors show how acid ceramidase and its enzymatic product sphingosine enable multivesicular bodies to function as an anti-viral mechanism.
Judith Lang +30 more
doaj +1 more source
The clinical spectrum of SMA‐PME and in vitro normalization of its cellular ceramide profile
Objective The objectives of this study were to define the clinical and biochemical spectrum of spinal muscular atrophy with progressive myoclonic epilepsy (SMA‐PME) and to determine if aberrant cellular ceramide accumulation could be normalized by enzyme
Michelle M. Lee +16 more
doaj +1 more source
Modifying Acid Ceramidase Inhibitors to Encourage Cancer Cell Apoptosis
In 2020 alone 1.9 million new cases of cancer were diagnosed. This challenging disease has a multitude of treatments that all hope to cure the cancer completely without recurrence.
Lang, Emma
core
Catalytic Mechanism of Eukaryotic Neutral Ceramidase [PDF]
Neutral ceramidases are key enzymes of sphingolipid metabolism that hydrolyze the fatty acyl/sphingosine amide linkage of ceramide at neutral pH. In this issue of Structure, Airola et al. (2015) present the first crystal structure of human nCDase and show how complexation with phosphate supports a new catalytic mechanism for Zn-dependent amidases while
Malinina, Lucy, Brown, Rhoderick E.
openaire +2 more sources
Ceramidases catalyze the cleavage of ceramides into sphingosine and fatty acids. Previously, we reported on the use of the RBM14 fluorogenic ceramide analogs to determine acidic ceramidase activity.
Mireia Casasampere +12 more
doaj +1 more source

