SGPL1 (sphingosine phosphate lyase 1) modulates neuronal autophagy via phosphatidylethanolamine production
Sign Up to like & getrecommendations! Published in 2017 at "Autophagy"
DOI: 10.1080/15548627.2017.1291471
Abstract: ABSTRACT Macroautophagy/autophagy defects have been identified as critical factors underlying the pathogenesis of neurodegenerative diseases. The roles of the bioactive signaling lipid sphingosine-1-phosphate (S1P) and its catabolic enzyme SGPL1/SPL (sphingosine phosphate lyase 1) in autophagy… read more here.
Keywords: lc3; phosphate lyase; autophagy; sphingosine phosphate ... See more keywords
Elevated sphingosine-1-phosphate lyase leads to increased metabolism and reduced survival in adrenocortical carcinoma.
Sign Up to like & getrecommendations! Published in 2023 at "European journal of endocrinology"
DOI: 10.1093/ejendo/lvac007
Abstract: OBJECTIVE Adrenocortical carcinomas (ACCs) are invasive tumours arising in the adrenal cortex, and steroidogenic tumours are associated with worse prognostic outcomes. Loss-of-function mutations in sphingosine-1-phosphate lyase (SGPL1) cause primary adrenal insufficiency and as a key… read more here.
Keywords: phosphate lyase; sphingosine phosphate; sgpl1; patient survival ... See more keywords
Fifty years of lyase and a moment of truth: sphingosine phosphate lyase from discovery to disease[S]
Sign Up to like & getrecommendations! Published in 2019 at "Journal of Lipid Research"
DOI: 10.1194/jlr.s091181
Abstract: Sphingosine phosphate lyase (SPL) is the final enzyme in the sphingolipid degradative pathway, catalyzing the irreversible cleavage of long-chain base phosphates (LCBPs) to yield a long-chain aldehyde and ethanolamine phosphate (EP). SPL guards the sole… read more here.
Keywords: phosphate; spl; discovery disease; sphingosine phosphate ... See more keywords
Insights From Long-term Follow-up of a Girl With Adrenal Insufficiency and Sphingosine-1-Phosphate Lyase Deficiency
Sign Up to like & getrecommendations! Published in 2022 at "Journal of the Endocrine Society"
DOI: 10.1210/jendso/bvac020
Abstract: Abstract Introduction Sphingosine-1-phosphate lyase (SGPL1) insufficiency syndrome (SPLIS) is a multisystemic disorder which, in the main, incorporates steroid-resistant nephrotic syndrome and primary adrenal insufficiency (PAI). Case Presentation We present a young girl with a novel… read more here.
Keywords: insufficiency; phosphate lyase; adrenal insufficiency; sgpl1 ... See more keywords
Neurological Consequences of Sphingosine Phosphate Lyase Insufficiency
Sign Up to like & getrecommendations! Published in 2022 at "Frontiers in Cellular Neuroscience"
DOI: 10.3389/fncel.2022.938693
Abstract: In 2017, an inborn error of metabolism caused by recessive mutations in SGPL1 was discovered. The disease features steroid-resistant nephrotic syndrome, adrenal insufficiency, and neurological defects. The latter can include sensorineural hearing loss, cranial nerve… read more here.
Keywords: spl; insufficiency; neurological consequences; phosphate lyase ... See more keywords