| Deciphering pathophysiological mechanisms underlying cystathionine beta-synthase-deficient homocystinuria using targeted metabolomics, liver proteomics, sphingolipidomics and analysis of mitochondrial function. | Deciphering pathophysiological mechanisms underlying cystathionine beta-synthase-deficient homocystinuria using targeted metabolomics, liver proteomics, sphingolipidomics and analysis of mitochondrial function.
Majtan T, Olsen T, Sokolova J, Krijt J, Křížková M, Ida T, Ditrói T, Hansikova H, Vit O, Petrak J, Kuchař L, Kruger WD, Nagy P, Akaike T, Kožich V., Free PMC Article | 06/25/2024 |
| KLF10/CBS increases the sensitivity of gastric carcinoma cells to methionine restriction by promoting sulfur transfer pathway. | KLF10/CBS increases the sensitivity of gastric carcinoma cells to methionine restriction by promoting sulfur transfer pathway.
Xin L, Liu ZY, Liu CX, Sheng J, Zhou Q, Yuan YW, Yue ZQ, Lu H, Gan JH. | 04/11/2024 |
| A role for the cystathionine-beta-synthase /H2S axis in astrocyte dysfunction in the aging brain. | A role for the cystathionine-β-synthase /H(2)S axis in astrocyte dysfunction in the aging brain.
Dey A, Pramanik PK, Dwivedi SKD, Neizer-Ashun F, Kiss T, Ganguly A, Rice H, Mukherjee P, Xu C, Ahmad M, Csiszar A, Bhattacharya R., Free PMC Article | 12/20/2023 |
| Activating transcription factor (ATF) 6 upregulates cystathionine beta synthetase (CBS) expression and hydrogen sulfide (H2S) synthesis to ameliorate liver metabolic damage. | Activating transcription factor (ATF) 6 upregulates cystathionine β synthetase (CBS) expression and hydrogen sulfide (H(2)S) synthesis to ameliorate liver metabolic damage.
Dong B, Sun Y, Cheng B, Xue Y, Li W, Sun X., Free PMC Article | 11/29/2023 |
| Homocysteine metabolites inhibit autophagy, elevate amyloid beta, and induce neuropathy by impairing Phf8/H4K20me1-dependent epigenetic regulation of mTOR in cystathionine beta-synthase-deficient mice. | Homocysteine metabolites inhibit autophagy, elevate amyloid beta, and induce neuropathy by impairing Phf8/H4K20me1-dependent epigenetic regulation of mTOR in cystathionine β-synthase-deficient mice.
Witucki Ł, Jakubowski H. | 11/16/2023 |
| Cystine/Glutamate Xc[-] Antiporter Induction Compensates for Transsulfuration Pathway Repression by 2,3,7,8-Tetrachlorodibenzo-p-dioxin (TCDD) to Ensure Cysteine for Hepatic Glutathione Biosynthesis. | Cystine/Glutamate Xc(-) Antiporter Induction Compensates for Transsulfuration Pathway Repression by 2,3,7,8-Tetrachlorodibenzo-p-dioxin (TCDD) to Ensure Cysteine for Hepatic Glutathione Biosynthesis.
Orlowska K, Fling RR, Nault R, Schilmiller AL, Zacharewski TR., Free PMC Article | 06/21/2023 |
| Cystathionine beta-synthase gene inactivation dysregulates major urinary protein biogenesis and impairs sexual signaling in mice. | Cystathionine β-synthase gene inactivation dysregulates major urinary protein biogenesis and impairs sexual signaling in mice.
Bretes E, Wróblewski J, Wyszczelska-Rokiel M, Jakubowski H. | 10/1/2022 |
| Cystathionine beta-synthase as novel endogenous regulator of lymphangiogenesis via modulating VEGF receptor 2 and 3. | Cystathionine β-synthase as novel endogenous regulator of lymphangiogenesis via modulating VEGF receptor 2 and 3.
Hatami N, Büttner C, Bock F, Simfors S, Musial G, Reis A, Cursiefen C, Clahsen T., Free PMC Article | 10/1/2022 |
| Derangement of hepatic polyamine, folate, and methionine cycle metabolism in cystathionine beta-synthase-deficient homocystinuria in the presence and absence of treatment: Possible implications for pathogenesis. | Derangement of hepatic polyamine, folate, and methionine cycle metabolism in cystathionine beta-synthase-deficient homocystinuria in the presence and absence of treatment: Possible implications for pathogenesis.
Maclean KN, Jiang H, Phinney WN, Mclagan BM, Roede JR, Stabler SP. | 08/14/2021 |
| Analysis of differential neonatal lethality in cystathionine beta-synthase deficient mouse models using metabolic profiling. | Analysis of differential neonatal lethality in cystathionine β-synthase deficient mouse models using metabolic profiling.
Gupta S, Wang L, Slifker MJ, Cai KQ, Maclean KN, Wasek B, Bottiglieri T, Kruger WD., Free PMC Article | 07/24/2021 |
| CRY1-CBS binding regulates circadian clock function and metabolism. | CRY1-CBS binding regulates circadian clock function and metabolism.
Cal-Kayitmazbatir S, Kulkoyluoglu-Cotul E, Growe J, Selby CP, Rhoades SD, Malik D, Oner H, Asimgil H, Francey LJ, Sancar A, Kruger WD, Hogenesch JB, Weljie A, Anafi RC, Kavakli IH., Free PMC Article | 06/19/2021 |
| Dysbiotic 1-carbon metabolism in cardiac muscle remodeling. | Dysbiotic 1-carbon metabolism in cardiac muscle remodeling.
George AK, Singh M, Pushpakumar S, Homme RP, Hardin SJ, Tyagi SC., Free PMC Article | 12/19/2020 |
| The Cbs Locus Affects the Expression of Senescence Markers and mtDNA Copy Number, but not Telomere Dynamics in Mice. | The Cbs Locus Affects the Expression of Senescence Markers and mtDNA Copy Number, but not Telomere Dynamics in Mice.
Utyro O, Perła-Kaján J, Jakubowski H., Free PMC Article | 12/19/2020 |
| Analysis of the Qatari R336C cystathionine beta-synthase protein in mice. | Analysis of the Qatari R336C cystathionine β-synthase protein in mice.
Gupta S, Gallego-Villar L, Wang L, Lee HO, Nasrallah G, Al-Dewik N, Häberle J, Thöny B, Blom HJ, Ben-Omran T, Kruger WD., Free PMC Article | 09/19/2020 |
| Sex affects N-homocysteinylation at lysine residue 212 of albumin in mice. | Sex affects N-homocysteinylation at lysine residue 212 of albumin in mice.
Sikora M, Marczak Ł, Perła-Kajan J, Jakubowski H., Free PMC Article | 09/19/2020 |
| we demonstrated that CBS overdosage underpins the DS-related recognition memory deficit and that both CBS and DYRK1A interact to control accurate memory processes in DS. | Cbs overdosage is necessary and sufficient to induce cognitive phenotypes in mouse models of Down syndrome and interacts genetically with Dyrk1a.
Marechal D, Brault V, Leon A, Martin D, Lopes Pereira P, Loaëc N, Birling MC, Friocourt G, Blondel M, Herault Y. | 03/14/2020 |
| HHcy dismantles BM-EPCs function and bone blood flow through the hyper-methylation of the CBS promoter in high methione fed mice. | Hyperhomocysteinemia induced endothelial progenitor cells dysfunction through hyper-methylation of CBS promoter.
Behera J, Tyagi SC, Tyagi N., Free PMC Article | 11/30/2019 |
| A blockade of HDAC3 inhibition in CBS(+/-) mice by HDAC activator ITSA-1, led to the remodeling of histone landscapes in the genome and thereby attenuated histone acetylation-dependent inflammatory signaling. | Hydrogen Sulfide Promotes Bone Homeostasis by Balancing Inflammatory Cytokine Signaling in CBS-Deficient Mice through an Epigenetic Mechanism.
Behera J, Kelly KE, Voor MJ, Metreveli N, Tyagi SC, Tyagi N., Free PMC Article | 11/9/2019 |
| CBS was selectively downregulated in both diet and gene-induced obesity models. | Specific downregulation of cystathionine β-synthase expression in the kidney during obesity.
Liu M, Deng M, Su J, Lin Y, Jia Z, Peng K, Wang F, Yang T., Free PMC Article | 09/7/2019 |
| Functional studies revealed that NaHS administration improved muscle fatigability in CBS(+/-) mice. In conclusion, our work provides evidence that NaHS is beneficial in mitigating HHcy-mediated skeletal injury incited by oxidative/ER stress responses. | Hydrogen sulfide alleviates hyperhomocysteinemia-mediated skeletal muscle atrophy via mitigation of oxidative and endoplasmic reticulum stress injury.
Majumder A, Singh M, Behera J, Theilen NT, George AK, Tyagi N, Metreveli N, Tyagi SC., Free PMC Article | 07/6/2019 |
| Data suggest that impaired cystathionine beta-synthase-hydrogen sulfide axis may contribute to the pathogenesis of Parkinson's disease, and that modulation of this axis may become a novel therapeutic approach for Parkinson's disease. | Impaired CBS-H(2)S signaling axis contributes to MPTP-induced neurodegeneration in a mouse model of Parkinson's disease.
Yuan YQ, Wang YL, Yuan BS, Yuan X, Hou XO, Bian JS, Liu CF, Hu LF. | 07/14/2018 |
| results suggested that reduced cystathionine beta-synthase/hydrogen sulfide AMP-activated protein kinase (CBS-H2S-AMPK) cascade activity contributed to microglia-mediated neuroinflammation following stroke. | The cystathionine β-synthase/hydrogen sulfide pathway contributes to microglia-mediated neuroinflammation following cerebral ischemia.
Zhang M, Wu X, Xu Y, He M, Yang J, Li J, Li Y, Ao G, Cheng J, Jia J. | 06/9/2018 |
| Results indicate that activation of the cystathionine-beta-synthase (CBS) and its product hydrogen sulfide (CBS/H2S axis promotes colon carcinogenesis. | Upregulation of Cystathionine-β-Synthase in Colonic Epithelia Reprograms Metabolism and Promotes Carcinogenesis.
Phillips CM, Zatarain JR, Nicholls ME, Porter C, Widen SG, Thanki K, Johnson P, Jawad MU, Moyer MP, Randall JW, Hellmich JL, Maskey M, Qiu S, Wood TG, Druzhyna N, Szczesny B, Módis K, Szabo C, Chao C, Hellmich MR., Free PMC Article | 12/2/2017 |
| showed that N-homocysteinylation of collagen in Cbs(-/-) mice impairs its cross-linking | N-Homocysteinylation impairs collagen cross-linking in cystathionine β-synthase-deficient mice: a novel mechanism of connective tissue abnormalities.
Perła-Kajan J, Utyro O, Rusek M, Malinowska A, Sitkiewicz E, Jakubowski H. | 09/23/2017 |
| the findings of this study indicate that a deficiency in 3MST does not significantly affect endotoxemia, while a deficiency in CBS or CSE slightly ameliorates the outcome of LPS-induced endotoxemia in vivo. | Effect of endotoxemia in mice genetically deficient in cystathionine-γ-lyase, cystathionine-β-synthase or 3-mercaptopyruvate sulfurtransferase.
Ahmad A, Gerö D, Olah G, Szabo C., Free PMC Article | 03/18/2017 |