9 results for "Kenneth Gable"

P053: Genotype phenotype correlations Inform therapeutic approach in serine palmitoyltransferase (SPT)-related disorders

Robert Thompson; Yi Gong; Kenneth Gable et al.

Genetics in Medicine Open, Vol. 3 · 2025 · English · DOAJ Scopus

Tsc10p and FVT1: topologically distinct short-chain reductases required for long-chain base synthesis in yeast and mammals

Sita D. Gupta; Kenneth Gable; Gongshe Han et al.

Journal of Lipid Research, Vol. 50 No. 8 · 2009 · English · DOAJ Scopus

In yeast, Tsc10p catalyzes reduction of 3-ketosphinganine to dihydrosphingosine. In mammals, it has been proposed that this reaction is catalyzed by FVT1, which despite limited homology and a different predicted topology, can replace Tsc10p in yeast. Silencing of FVT1 revealed a direct correlation…

Erratum: Use of isotopically labeled substrates reveals kinetic differences between human and bacterial serine palmitoyltransferase

Peter J. Harrison; Kenneth Gable; Niranjanakumari Somashekarappa et al.

Journal of Lipid Research, Vol. 60 No. 8 · 2019 · English · DOAJ Scopus

Use of isotopically labeled substrates reveals kinetic differences between human and bacterial serine palmitoyltransferase

Peter J. Harrison; Kenneth Gable; Niranjanakumari Somashekarappa et al.

Journal of Lipid Research, Vol. 60 No. 5 · 2019 · English · DOAJ Scopus

Isotope labels are frequently used tools to track metabolites through complex biochemical pathways and to discern the mechanisms of enzyme-catalyzed reactions. Isotopically labeled l-serine is often used to monitor the activity of the first enzyme in sphingolipid biosynthesis, serine…

A Neurotoxic Glycerophosphocholine Impacts PtdIns-4, 5-Bisphosphate and TORC2 Signaling by Altering Ceramide Biosynthesis in Yeast

Michael A. Kennedy; Kenneth Gable; Karolina Niewola-Staszkowska et al.

PLoS Genetics, Vol. 10 No. 1 · 2014 · English · PLOS Scopus

Unbiased lipidomic approaches have identified impairments in glycerophosphocholine second messenger metabolism in patients with Alzheimer's disease. Specifically, we have shown that amyloid-β42 signals the intraneuronal accumulation of PC(O-16:0/2:0) which is associated with neurotoxicity. Similar…

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Collaborative regulation of yeast SPT-Orm2 complex by phosphorylation and ceramide

Tian Xie; Feitong Dong; Gongshe Han et al.

Cell Reports, Vol. 43 No. 2 · 2024 · English · DOAJ Scopus

Summary: The homeostatic regulation of serine palmitoyltransferase (SPT) activity in yeast involves N-terminal phosphorylation of Orm proteins, while higher eukaryotes lack these phosphorylation sites. Although recent studies have indicated a conserved ceramide-mediated feedback inhibition of the…

TORC1 Inhibits GSK3-Mediated Elo2 Phosphorylation to Regulate Very Long Chain Fatty Acid Synthesis and Autophagy

Christine Zimmermann; Aline Santos; Kenneth Gable et al.

Cell Reports, Vol. 18 No. 8 · 2017 · English · DOAJ Scopus

TORC1 Inhibits GSK3-Mediated Elo2 Phosphorylation to Regulate Very Long Chain Fatty Acid Synthesis and Autophagy

Christine Zimmermann; Aline Santos; Kenneth Gable et al.

Cell Reports, Vol. 5 No. 4 · 2013 · English · DOAJ Scopus

Very long chain fatty acids (VLCFAs) are essential fatty acids with multiple functions, including ceramide synthesis. Although the components of the VLCFA biosynthetic machinery have been elucidated, how their activity is regulated to meet the cell’s metabolic demand remains unknown. The goal of…