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Succinyl-CoA:3-oxoacid coenzyme A transferase (SCOT) deficiency: A rare and potentially fatal metabolic disease.
Grünert SC, Foster W, Schumann A, Lund A, Pontes C, Roloff S, Weinhold N, Yue WW, AlAsmari A, Obaid OA, Faqeih EA, Stübbe L, Yamamoto R, Gemperle-Britschgi C, Walter M, Spiekerkoetter U, Mackinnon S, Sass JO. Grünert SC, et al. Biochimie. 2021 Apr;183:55-62. doi: 10.1016/j.biochi.2021.02.003. Epub 2021 Feb 14. Biochimie. 2021. PMID: 33596448
Inborn errors of ketone body utilization.
Hori T, Yamaguchi S, Shinkaku H, Horikawa R, Shigematsu Y, Takayanagi M, Fukao T. Hori T, et al. Pediatr Int. 2015;57(1):41-8. doi: 10.1111/ped.12585. Pediatr Int. 2015. PMID: 25559898 Review.
Succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency and mitochondrial acetoacetyl-CoA thiolase (beta-ketothiolase or T2) deficiency are classified as autosomal recessive disorders of ketone body utilization characteriz
Succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency and mitochondrial acetoace
Ketone body metabolism and its defects.
Fukao T, Mitchell G, Sass JO, Hori T, Orii K, Aoyama Y. Fukao T, et al. J Inherit Metab Dis. 2014 Jul;37(4):541-51. doi: 10.1007/s10545-014-9704-9. Epub 2014 Apr 8. J Inherit Metab Dis. 2014. PMID: 24706027 Review.
Patients with HL deficiency can develop hypoglycemic crises and neurological symptoms even in adolescents and adults. Succinyl-CoA-3-oxoacid CoA transferase (SCOT) deficiency and beta-ketothiolase (T2) deficiency are two defects in ketolysis. Permanent ketosis is pa …
Patients with HL deficiency can develop hypoglycemic crises and neurological symptoms even in adolescents and adults. Succinyl-CoA-3-oxoacid …
Defective muscle ketone body oxidation disrupts BCAA catabolism by altering mitochondrial branched-chain aminotransferase.
Mechchate H, Abdualkader AM, Bernacchi JB, Gopal K, Tabatabaei Dakhili SA, Yang K, Greenwell AA, Kong X, Crawford PA, Al Batran R. Mechchate H, et al. Am J Physiol Endocrinol Metab. 2023 May 1;324(5):E425-E436. doi: 10.1152/ajpendo.00206.2022. Epub 2023 Mar 29. Am J Physiol Endocrinol Metab. 2023. PMID: 36989424
Skeletal muscle is an important site of ketone body oxidation that occurs through a series of reactions requiring the enzyme succinyl-CoA:3-ketoacid-CoA transferase (SCOT/Oxct1). We have previously shown that deleting SCOT in the skeletal …
Skeletal muscle is an important site of ketone body oxidation that occurs through a series of reactions requiring the enzyme succinyl
Beneficial effects on kidney during treatment with sodium-glucose cotransporter 2 inhibitors: proposed role of ketone utilization.
Hattori Y. Hattori Y. Heart Fail Rev. 2021 Jul;26(4):947-952. doi: 10.1007/s10741-020-10065-7. Epub 2021 Jan 6. Heart Fail Rev. 2021. PMID: 33404998 Review.
Modestly elevated circulating levels of the ketone beta-hydroxybutyrate (betaOHB) during treatment with sodium-glucose cotransporter 2 (SGLT2) inhibitors cause different beneficial effects on organs and cells, depending on the succinyl-CoA:3-ketoacid
Modestly elevated circulating levels of the ketone beta-hydroxybutyrate (betaOHB) during treatment with sodium-glucose cotransporter 2 (SGLT …
Insulin resistance and heart failure during treatment with sodium glucose cotransporter 2 inhibitors: proposed role of ketone utilization.
Hattori Y. Hattori Y. Heart Fail Rev. 2020 May;25(3):403-408. doi: 10.1007/s10741-020-09921-3. Heart Fail Rev. 2020. PMID: 31960270 Review.
Modestly elevated circulating beta-hydroxybutyrate (betaOHB) during treatment with SGLT2 inhibitors causes different beneficial effects on organs and cells, depending on succinyl-CoA:3-ketoacid CoA transferase (SCOT) levels. In the heart, …
Modestly elevated circulating beta-hydroxybutyrate (betaOHB) during treatment with SGLT2 inhibitors causes different beneficial effects on o …
A Rare Cause of Life-Threatening Ketoacidosis: Novel Compound Heterozygous OXCT1 Mutations Causing Succinyl-CoA:3-Ketoacid CoA Transferase Deficiency.
Kim YA, Kim SH, Cheon CK, Kim YM. Kim YA, et al. Yonsei Med J. 2019 Mar;60(3):308-311. doi: 10.3349/ymj.2019.60.3.308. Yonsei Med J. 2019. PMID: 30799594 Free PMC article.
Succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency is a rare inborn error of ketone body utilization, characterized by episodic or permanent ketosis. SCOT deficiency is caused by mutations in the OXCT1 g
Succinyl-CoA:3-ketoacid CoA transferase (SCOT) deficiency is a rare inborn error of
Pharmacological Inhibition of Succinyl Coenzyme A:3-Ketoacid Coenzyme A Transferase Alleviates the Progression of Diabetic Cardiomyopathy.
Greenwell AA, Tabatabaei Dakhili SA, Wagg CS, Saed CT, Chan JSF, Yang K, Mangra-Bala IA, Stenlund MJ, Eaton F, Gopal K, Dyck JRB, Lopaschuk GD, Ussher JR. Greenwell AA, et al. J Am Heart Assoc. 2024 Apr 2;13(7):e032697. doi: 10.1161/JAHA.123.032697. Epub 2024 Mar 27. J Am Heart Assoc. 2024. PMID: 38533954 Free article. No abstract available.
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