Kenneth Gable

Uniformed Services University of the Health Sciences — United States

Biochemistry, Genetics and Molecular Biology · Life Sciences

27h-index2.5kcitations50works3.02yr avg
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Research Topics

Sphingolipid Metabolism and Signaling(16), Cellular transport and secretion(12), Endoplasmic Reticulum Stress and Disease(11), Lipid Membrane Structure and Behavior(9), Amyotrophic Lateral Sclerosis Research(7)

Publications50 total

Characterization of novel and recurrent <i>SPTLC2</i> variants in childhood-onset amyotrophic lateral sclerosis: Insights into sphingolipid dysregulation
Journal of Neuromuscular Diseases·2025Open Access
P053: Genotype phenotype correlations Inform therapeutic approach in serine palmitoyltransferase (SPT)-related disorders
Genetics in Medicine Open·2025Open Access
Childhood Onset Amyotrophic Lateral Sclerosis Associated with SPTLC2 Gain-of-Function Pathogenic Variants: Clinical, Genetic, and Biochemical Insights (P4-8.001)
Neurology·2024
Collaborative regulation of yeast SPT-Orm2 complex by phosphorylation and ceramide
Cell Reports·2024· 12 citedOpen Access
Recurrent de novo<i>SPTLC2</i>variant causes childhood-onset amyotrophic lateral sclerosis (ALS) by excess sphingolipid synthesis
Journal of Neurology Neurosurgery & Psychiatry·2023· 19 citedOpen Access
Recurrent<i>de-novo gain-of-function</i>mutation in<i>SPTLC2</i>confirms dysregulated sphingolipid production to cause juvenile amyotrophic lateral sclerosis
Journal of Neurology Neurosurgery & Psychiatry·2023· 13 citedOpen Access
Mechanism of sphingolipid homeostasis revealed by structural analysis of <i>Arabidopsis</i> SPT-ORM1 complex
Science Advances·2023· 18 citedOpen Access
<i>SPTSSA</i> variants alter sphingolipid synthesis and cause a complex hereditary spastic paraplegia
Brain·2023· 33 citedOpen Access
The <i>SPTLC1</i> p.S331 mutation bridges sensory neuropathy and motor neuron disease and has implications for treatment
Neuropathology and Applied Neurobiology·2022· 12 citedOpen Access
Childhood amyotrophic lateral sclerosis caused by excess sphingolipid synthesis
Nature Medicine·2021· 149 citedOpen Access
Structural insights into the regulation of human serine palmitoyltransferase complexes
The FASEB Journal·2021
SPTLC1 Mutations Associated with Early Onset Amyotrophic Lateral Sclerosis Impair ORMDL Regulation
The FASEB Journal·2021
Structural insights into the regulation of human serine palmitoyltransferase complexes
Nature Structural & Molecular Biology·2021· 98 citedOpen Access
SPTLC1 Mutations Associated with Early Onset Amyotrophic Lateral Sclerosis
The FASEB Journal·2020· 3 cited
Erratum: Use of isotopically labeled substrates reveals kinetic differences between human and bacterial serine palmitoyltransferase
Journal of Lipid Research·2019· 1 citedOpen Access
Use of isotopically labeled substrates reveals kinetic differences between human and bacterial serine palmitoyltransferase
Journal of Lipid Research·2019· 9 citedOpen Access
The ORMDL/Orm–serine palmitoyltransferase (SPT) complex is directly regulated by ceramide: Reconstitution of SPT regulation in isolated membranes
Journal of Biological Chemistry·2019· 104 citedOpen Access
The ORMs interact with transmembrane domain 1 of Lcb1 and regulate serine palmitoyltransferase oligomerization, activity and localization
Biochimica et Biophysica Acta (BBA) - Molecular and Cell Biology of Lipids·2018· 40 cited
Yeast mating: Ceramide acyl chain length matters?
Cell Cycle·2017· 1 citedOpen Access
Regulation of sphingolipid biosynthesis by the morphogenesis checkpoint kinase Swe1.
Journal of Biological Chemistry·2017· 4 citedOpen Access
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Frequent Co-authors

Teresa Dunn(39), Sita D. Gupta(21), Gongshe Han(16), Niranjanakumari Somashekarappa(14), Troy Beeler(10), Jeffrey M. Harmon(9), Payam Mohassel(7), Sandra Donkervoort(6), Carsten G. Bönnemann(6), Museer A. Lone(4), Chia‐Hsueh Lee(4), Thorsten Hornemann(4), Richard L. Proia(4), A. Reghan Foley(3), Zoe Piccus(3), Claire E. Le Pichon(3), Edgar B. Cahoon(3), Dagmar Bačíková(3), Peter J. Harrison(3), Dominic J. Campopiano(3)