Publications by authors named "Kenneth Gable"

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Characterization of novel and recurrent variants in childhood-onset amyotrophic lateral sclerosis: Insights into sphingolipid dysregulation.
Xiaona Fu, Kenneth Gable, Sita D Gupta, KaiLi Zhang, Bingbing Jia

J Neuromuscul Dis· August 2025

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Recurrent mutation in confirms dysregulated sphingolipid production to cause juvenile amyotrophic lateral sclerosis.
Maike F Dohrn, Danique Beijer, Museer A Lone, Elif Bayraktar, Piraye Oflazer, Kenneth Gable

J Neurol Neurosurg Psychiatry· February 2024

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Collaborative regulation of yeast SPT-Orm2 complex by phosphorylation and ceramide.
Tian Xie, Feitong Dong, Gongshe Han, Xinyue Wu, Peng Liu, Kenneth Gable

Cell Rep· February 2024

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Recurrent de novo variant causes childhood-onset amyotrophic lateral sclerosis (ALS) by excess sphingolipid synthesis.
Safoora B Syeda, Museer A Lone, Payam Mohassel, Sandra Donkervoort, Pinki Munot, Kenneth Gable

J Neurol Neurosurg Psychiatry· January 2024

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SPTSSA variants alter sphingolipid synthesis and cause a complex hereditary spastic paraplegia.
Siddharth Srivastava, Hagar Mor Shaked, Kenneth Gable, Sita D Gupta, Xueyang Pan

Brain· April 2023

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Mechanism of sphingolipid homeostasis revealed by structural analysis of SPT-ORM1 complex.
Peng Liu, Tian Xie, Xinyue Wu, Gongshe Han, Sita D Gupta, Kenneth Gable

Sci Adv· March 2023

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The SPTLC1 p.S331 mutation bridges sensory neuropathy and motor neuron disease and has implications for treatment.
Chiara Fiorillo, Giovanna Capodivento, Alessandro Geroldi, Stefano Tozza, Isabella Moroni, Kenneth Gable

Neuropathol Appl Neurobiol· December 2022

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Childhood amyotrophic lateral sclerosis caused by excess sphingolipid synthesis.
Payam Mohassel, Sandra Donkervoort, Museer A Lone, Matthew Nalls, Kenneth Gable

Nat Med· July 2021

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Structural insights into the regulation of human serine palmitoyltransferase complexes.
Yingdi Wang, Yiming Niu, Zhe Zhang, Kenneth Gable, Sita D Gupta

Nat Struct Mol Biol· March 2021

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Erratum: Use of isotopically labeled substrates reveals kinetic differences between human and bacterial serine palmitoyltransferase.
Peter J Harrison, Kenneth Gable, Niranjanakumari Somashekarappa, Van Kelly, David J Clarke

J Lipid Res· August 2019

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Yeast mating: Ceramide acyl chain length matters?
Teresa M Dunn, Kenneth Gable, Gongshe Han

Cell Cycle· June 2019

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Use of isotopically labeled substrates reveals kinetic differences between human and bacterial serine palmitoyltransferase.
Peter J Harrison, Kenneth Gable, Niranjanakumari Somashekarappa, Van Kelly, David J Clarke

J Lipid Res· May 2019

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The ORMs interact with transmembrane domain 1 of Lcb1 and regulate serine palmitoyltransferase oligomerization, activity and localization.
Gongshe Han, Sita D Gupta, Kenneth Gable, Dagmar Bacikova, Nivedita Sengupta

Biochim Biophys Acta Mol Cell Biol Lipids· March 2019

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The ORMDL/Orm-serine palmitoyltransferase (SPT) complex is directly regulated by ceramide: Reconstitution of SPT regulation in isolated membranes.
Deanna L Davis, Kenneth Gable, John Suemitsu, Teresa M Dunn, Binks W Wattenberg

J Biol Chem· March 2019

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Regulation of sphingolipid biosynthesis by the morphogenesis checkpoint kinase Swe1.
Neha Chauhan, Gongshe Han, Niranjanakumari Somashekarappa, Kenneth Gable, Teresa Dunn

J Biol Chem· June 2017

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TORC1 Inhibits GSK3-Mediated Elo2 Phosphorylation to Regulate Very Long Chain Fatty Acid Synthesis and Autophagy.
Christine Zimmermann, Aline Santos, Kenneth Gable, Sharon Epstein, Charulatha Gururaj

Cell Rep· February 2017

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Ectopic expression of ceramide synthase 2 in neurons suppresses neurodegeneration induced by ceramide synthase 1 deficiency.
Stefka D Spassieva, Xiaojie Ji, Ye Liu, Kenneth Gable, Jacek Bielawski

Proc Natl Acad Sci U S A· May 2016

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Regulation of Sphingolipid Biosynthesis by the Morphogenesis Checkpoint Kinase Swe1.
Neha Chauhan, Gongshe Han, Niranjanakumari Somashekarappa, Kenneth Gable, Teresa Dunn

J Biol Chem· January 2016

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A Signaling Lipid Associated with Alzheimer's Disease Promotes Mitochondrial Dysfunction.
Michael A Kennedy, Tia C Moffat, Kenneth Gable, Suriakarthiga Ganesan, Karolina Niewola-Staszkowska

Sci Rep· January 2016

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Elevation of 20-carbon long chain bases due to a mutation in serine palmitoyltransferase small subunit b results in neurodegeneration.
Lihong Zhao, Stefka Spassieva, Kenneth Gable, Sita D Gupta, Lan-Ying Shi

Proc Natl Acad Sci U S A· October 2015

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Expression of the ORMDLS, modulators of serine palmitoyltransferase, is regulated by sphingolipids in mammalian cells.
Sita D Gupta, Kenneth Gable, Aikaterini Alexaki, Panagiotis Chandris, Richard L Proia

J Biol Chem· January 2015

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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, Susana Abreu, Anne Johnston

PLoS Genet· January 2014

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TORC1 inhibits GSK3-mediated Elo2 phosphorylation to regulate very long chain fatty acid synthesis and autophagy.
Christine Zimmermann, Aline Santos, Kenneth Gable, Sharon Epstein, Charulatha Gururaj

Cell Rep· November 2013

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Topological and functional characterization of the ssSPTs, small activating subunits of serine palmitoyltransferase.
Jeffrey M Harmon, Dagmar Bacikova, Kenneth Gable, Sita D Gupta, Gongshe Han

J Biol Chem· April 2013

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Sphingolipids in the root play an important role in regulating the leaf ionome in Arabidopsis thaliana.
Dai-Yin Chao, Kenneth Gable, Ming Chen, Ivan Baxter, Charles R Dietrich

Plant Cell· March 2011

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