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Kenneth Gable

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Articles 33
Citations 931
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Recent Articles
1.
Xie T, Dong F, Han G, Wu X, Liu P, Zhang Z, et al.
Cell Rep . 2024 Jan; 43(2):113717. PMID: 38285738
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...
2.
Dohrn M, Beijer D, Lone M, Bayraktar E, Oflazer P, Orbach R, et al.
J Neurol Neurosurg Psychiatry . 2023 Dec; 95(3):201-205. PMID: 38041684
Background: Amyotrophic lateral sclerosis (ALS) leads to paralysis and death by progressive degeneration of motor neurons. Recently, specific mutations in were identified in patients with juvenile form of ALS encodes...
3.
Syeda S, Lone M, Mohassel P, Donkervoort S, Munot P, Franca Jr M, et al.
J Neurol Neurosurg Psychiatry . 2023 Dec; 95(2):103-113. PMID: 38041679
Background: Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease of the upper and lower motor neurons with varying ages of onset, progression and pathomechanisms. Monogenic childhood-onset ALS, although rare, forms...
4.
Liu P, Xie T, Wu X, Han G, Gupta S, Zhang Z, et al.
Sci Adv . 2023 Mar; 9(13):eadg0728. PMID: 36989369
The serine palmitoyltransferase (SPT) complex catalyzes the first and rate-limiting step in sphingolipid biosynthesis in all eukaryotes. ORM/ORMDL proteins are negative regulators of SPT that respond to cellular sphingolipid levels....
5.
Srivastava S, Shaked H, Gable K, Gupta S, Pan X, Somashekarappa N, et al.
Brain . 2023 Jan; 146(4):1420-1435. PMID: 36718090
Sphingolipids are a diverse family of lipids with critical structural and signalling functions in the mammalian nervous system, where they are abundant in myelin membranes. Serine palmitoyltransferase, the enzyme that...
6.
Fiorillo C, Capodivento G, Geroldi A, Tozza S, Moroni I, Mohassel P, et al.
Neuropathol Appl Neurobiol . 2022 Jul; 48(7):e12842. PMID: 35904184
Aims: SPTLC1-related disorder is a late onset sensory-autonomic neuropathy associated with perturbed sphingolipid homeostasis which can be improved by supplementation with the serine palmitoyl-CoA transferase (SPT) substrate, l-serine. Recently, a...
7.
Mohassel P, Donkervoort S, Lone M, Nalls M, Gable K, Gupta S, et al.
Nat Med . 2021 Jun; 27(7):1197-1204. PMID: 34059824
Amyotrophic lateral sclerosis (ALS) is a progressive, neurodegenerative disease of the lower and upper motor neurons with sporadic or hereditary occurrence. Age of onset, pattern of motor neuron degeneration and...
8.
Wang Y, Niu Y, Zhang Z, Gable K, Gupta S, Somashekarappa N, et al.
Nat Struct Mol Biol . 2021 Feb; 28(3):240-248. PMID: 33558761
Sphingolipids are essential lipids in eukaryotic membranes. In humans, the first and rate-limiting step of sphingolipid synthesis is catalyzed by the serine palmitoyltransferase holocomplex, which consists of catalytic components (SPTLC1...
9.
Harrison P, Gable K, Somashekarappa N, Kelly V, Clarke D, Naismith J, et al.
J Lipid Res . 2019 Aug; 60(8):1489. PMID: 31371643
No abstract available.
10.
Harrison P, Gable K, Somashekarappa N, Kelly V, Clarke D, Naismith J, et al.
J Lipid Res . 2019 Feb; 60(5):953-962. PMID: 30792183
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...