-
Article
Open AccessSMPDL3b modulates insulin receptor signaling in diabetic kidney disease
Sphingomyelin phosphodiesterase acid-like 3b (SMPDL3b) is a lipid raft enzyme that regulates plasma membrane (PM) fluidity. Here we report that SMPDL3b excess, as observed in podocytes in diabetic kidney disea...
-
Article
Open AccessAltering the sphingolipid acyl chain composition prevents LPS/GLN-mediated hepatic failure in mice by disrupting TNFR1 internalization
The involvement of ceramide in death receptor-mediated apoptosis has been widely examined with most studies focusing on the role of ceramide generated from sphingomyelin hydrolysis. We now analyze the effect o...
-
Article
The metabolism and function of sphingolipids and glycosphingolipids
Sphingolipids and glycosphingolipids are emerging as major players in many facets of cell physiology and pathophysiology. We now present an overview of sphingolipid biochemistry and physiology, followed by a b...
-
Article
Elevation of lung surfactant phosphatidylcholine in mouse models of Sandhoff and of Niemann–Pick A disease
Summary: Sandhoff disease is caused by the defective activity of the lysosomal enzyme β-hexosaminidase, resulting in accumulation of the glycolipids, GA2 and GM2. Niemann–Pick A/B disease is caused by the defecti...
-
Article
The increased sensitivity of neurons with elevated glucocerebroside to neurotoxic agents can be reversed by imiglucerase
We have recently demonstrated that incubation of cultured rat hippocampal neurons with conduritol β-epoxide (CBE), an inhibitor of glucocerebrosidase, the enzyme defective in Gaucher disease, results in change...
-
Chapter
Nerve Cell Death Induced by Ca2+ Ionophores in Dissociated Hippocampal Cultures
Lasalocid (X-537A) is a polyether ionophore compound (Westly et al., 1970) that was isolated from streptomyces lasaliensis in 1951 (Berger et al., 1951). It is a lipid-soluble material which facilitates the passa...
