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111 results

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Page 1
Branched-chain amino acids in metabolic signalling and insulin resistance.
Lynch CJ, Adams SH. Lynch CJ, et al. Nat Rev Endocrinol. 2014 Dec;10(12):723-36. doi: 10.1038/nrendo.2014.171. Epub 2014 Oct 7. Nat Rev Endocrinol. 2014. PMID: 25287287 Free PMC article. Review.
Research on the role of individual and model-dependent differences in BCAA metabolism is needed, as several genes (BCKDHA, PPM1K, IVD and KLF15) have been designated as candidate genes for obesity and/or T2DM in humans, and distinct phenotypes of tissue-specific branched c …
Research on the role of individual and model-dependent differences in BCAA metabolism is needed, as several genes (BCKDHA, PPM1K, IVD …
BCKDH kinase promotes hepatic gluconeogenesis independent of BCKDHA.
Zhou F, Sheng C, Ma X, Li T, Ming X, Wang S, Tan J, Yang Y, Sun H, Lu J, Liu J, Deng R, Wang X, Zhou L. Zhou F, et al. Cell Death Dis. 2024 Oct 10;15(10):736. doi: 10.1038/s41419-024-07071-0. Cell Death Dis. 2024. PMID: 39389936 Free PMC article.
The rate limiting enzyme of BCAA catabolism branched-chain alpha-ketoacid dehydrogenase (BCKDH) is phosphorylated at E1alpha subunit (BCKDHA) by its kinase (BCKDK) and inactivated. Here, the liver-specific BCKDK or BCKDHA knockout mice displayed normal glucose toler …
The rate limiting enzyme of BCAA catabolism branched-chain alpha-ketoacid dehydrogenase (BCKDH) is phosphorylated at E1alpha subunit (BCK …
Impaired branched-chain amino acid (BCAA) catabolism during adipocyte differentiation decreases glycolytic flux.
Green CR, Alaeddine LM, Wessendorf-Rodriguez KA, Turner R, Elmastas M, Hover JD, Murphy AN, Ryden M, Mejhert N, Metallo CM, Wallace M. Green CR, et al. J Biol Chem. 2024 Dec;300(12):108004. doi: 10.1016/j.jbc.2024.108004. Epub 2024 Nov 16. J Biol Chem. 2024. PMID: 39551140 Free PMC article.
While lipid droplet accumulation is maintained in Bckdha-deficient adipocytes, they display a more fibroblast-like transcriptional signature. ...Decreased glycolytic flux emerges as the most distinct metabolic feature of 3T3-L1 Bckdha-deficient cells, accompanied by …
While lipid droplet accumulation is maintained in Bckdha-deficient adipocytes, they display a more fibroblast-like transcriptional si …
BCKDHA-BCKDHB digenic gene therapy restores metabolic homeostasis in two mouse models and a calf with classic maple syrup urine disease.
Wang J, Poskitt LE, Gallagher J, Puffenberger EG, Wynn RM, Shishodia G, Chuang DT, Beever J, Hardin DL, Brigatti KW, Baker WC, Gately R, Bertrand S, Rodrigues A, Benatti HR, Taghian T, Hall E, Prestigiacomo R, Liang J, Chen G, Zhou X, Ren L, Liu N, He R, Su Q, Xie J, Jiang Z, Gruntman A, Gray-Edwards H, Gao G, Strauss KA, Wang D. Wang J, et al. Sci Transl Med. 2025 Feb 26;17(787):eads0539. doi: 10.1126/scitranslmed.ads0539. Epub 2025 Feb 26. Sci Transl Med. 2025. PMID: 40009698
To develop a gene replacement therapy for MSUD, we designed a dual-function recombinant adeno-associated virus serotype 9 (rAAV9) vector to deliver codon-optimized BCKDHA and BCKDHB (rAAV9.hA-BiP-hB) to the liver, muscle, heart, and brain. rAAV9.hA-BiP-hB restored coexpres …
To develop a gene replacement therapy for MSUD, we designed a dual-function recombinant adeno-associated virus serotype 9 (rAAV9) vector to …
The pyruvate dehydrogenase kinase inhibitor dichloroacetate mitigates alcohol-induced hepatic inflammation and metabolic disturbances in mice.
Wu J, Huang E, McMullen MR, Singh V, Mrdjen M, Bellar A, Wang L, Welch N, Dasarathy J, Dasarathy S, Streem D, Brown JM, Nagy LE. Wu J, et al. Hepatol Commun. 2024 Nov 29;8(12):e0547. doi: 10.1097/HC9.0000000000000547. eCollection 2024 Dec 1. Hepatol Commun. 2024. PMID: 39621302 Free PMC article.
DCA blunted the ethanol-induced increase of BCKDHA, the rate-limiting enzyme in BCAA catabolism, and cytochrome P450 2E1. CONCLUSIONS: Ethanol-induced hepatic inflammatory responses and metabolic disturbances were prevented by DCA in mice, indicating the potential to devel …
DCA blunted the ethanol-induced increase of BCKDHA, the rate-limiting enzyme in BCAA catabolism, and cytochrome P450 2E1. CONCLUSIONS …
Sodium-glucose cotransporter 2 inhibitors-but not insulin-enhance renal branched-chain amino acid catabolism.
Sakamoto M, Hasuzawa N, Wang L, Tashiro K, Kitamura M, Iwata S, Wada N, Nomura S, Moriyama S, Gobaru M, Inoguchi Y, Nagayama A, Ashida K, Moriyama Y, Nomura M. Sakamoto M, et al. Front Endocrinol (Lausanne). 2025 Nov 7;16:1706838. doi: 10.3389/fendo.2025.1706838. eCollection 2025. Front Endocrinol (Lausanne). 2025. PMID: 41282284 Free PMC article. Clinical Trial.
To explore mechanisms, 10-week-old db/db mice received luseogliflozin (10 mg/kg/day, p.o.) or insulin glargine (10 U/day, s.c.) for 4 weeks; renal histology, mRNA and protein expression of key enzymes involved in BCAA catabolism, including branched-chain aminotransferase 2 (BCAT2 …
To explore mechanisms, 10-week-old db/db mice received luseogliflozin (10 mg/kg/day, p.o.) or insulin glargine (10 U/day, s.c.) for 4 weeks; …
The mitochondrial BCKD complex interacts with hepatic apolipoprotein E in cultured cells in vitro and mouse livers in vivo.
Rueter J, Rimbach G, Treitz C, Schloesser A, Lüersen K, Tholey A, Huebbe P. Rueter J, et al. Cell Mol Life Sci. 2023 Feb 7;80(3):59. doi: 10.1007/s00018-023-04706-x. Cell Mol Life Sci. 2023. PMID: 36749362 Free PMC article.
Proteomic analysis of coimmunoprecipitation products from APOE mouse liver extracts revealed 28 APOE-interacting candidate proteins, including branched-chain alpha-keto acid dehydrogenase (BCKD) complex subunit alpha (BCKDHA) and voltage-dependent anion-selective ch …
Proteomic analysis of coimmunoprecipitation products from APOE mouse liver extracts revealed 28 APOE-interacting candidate proteins, includi …
Genome-wide association and multi-trait analyses characterize the common genetic architecture of heart failure.
Levin MG, Tsao NL, Singhal P, Liu C, Vy HMT, Paranjpe I, Backman JD, Bellomo TR, Bone WP, Biddinger KJ, Hui Q, Dikilitas O, Satterfield BA, Yang Y, Morley MP, Bradford Y, Burke M, Reza N, Charest B; Regeneron Genetics Center; Judy RL, Puckelwartz MJ, Hakonarson H, Khan A, Kottyan LC, Kullo I, Luo Y, McNally EM, Rasmussen-Torvik LJ, Day SM, Do R, Phillips LS, Ellinor PT, Nadkarni GN, Ritchie MD, Arany Z, Cappola TP, Margulies KB, Aragam KG, Haggerty CM, Joseph J, Sun YV, Voight BF, Damrauer SM. Levin MG, et al. Nat Commun. 2022 Nov 14;13(1):6914. doi: 10.1038/s41467-022-34216-6. Nat Commun. 2022. PMID: 36376295 Free PMC article.
Colocalization, gene expression profiling, and Mendelian randomization provide convergent evidence for the roles of BCKDHA and circulating branch-chain amino acids in heart failure and cardiac structure. ...
Colocalization, gene expression profiling, and Mendelian randomization provide convergent evidence for the roles of BCKDHA and circul …
Three novel mutations of the BCKDHA, BCKDHB and DBT genes in Chinese children with maple syrup urine disease.
Yang J, Xiu J, Sun Y, Liu F, Shang X, Li G. Yang J, et al. J Pediatr Endocrinol Metab. 2021 Dec 10;35(3):303-312. doi: 10.1515/jpem-2021-0672. Print 2022 Mar 28. J Pediatr Endocrinol Metab. 2021. PMID: 34883003
BACKGROUND: Maple syrup urine disease (MSUD) is a rare metabolic autosomal recessive disorder caused by deficiency of the branched-chain alpha-ketoacid dehydrogenase complex. Mutations in the BCKDHA, BCKDHB and DBT genes are responsible for MSUD. This study presents the cl …
BACKGROUND: Maple syrup urine disease (MSUD) is a rare metabolic autosomal recessive disorder caused by deficiency of the branched-chain alp …
Maple syrup urine disease: it has come a long way.
Chuang DT. Chuang DT. J Pediatr. 1998 Mar;132(3 Pt 2):S17-23. doi: 10.1016/s0022-3476(98)70523-2. J Pediatr. 1998. PMID: 9546032 Review.
This led to the later cloning of cDNAs and genes for subunits of the human BCKD complex. Genetic heterogeneity in MSUD is now explained by the various mutations that occur in the E1 alpha, E1 beta, E2, and E3 loci of the BCKD complex. Recently, we foun …
This led to the later cloning of cDNAs and genes for subunits of the human BCKD complex. Genetic heterogeneity in MSUD is now explain …
111 results