L-Cystathionine (BioDeep_00000001337)

 

Secondary id: BioDeep_00000027581, BioDeep_00000227898, BioDeep_00000399916, BioDeep_00000400228, BioDeep_00000405277

natural product human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite BioNovoGene_Lab2019


代谢物信息卡片


(2S)-2-amino-4-{[(2R)-2-amino-2-carboxyethyl]sulfanyl}butanoic acid

化学式: C7H14N2O4S (222.0674244)
中文名称: L-胱硫醚, DL-胱硫醚, 胱硫醚
谱图信息: 最多检出来源 Homo sapiens(blood) 0.07%

Reviewed

Last reviewed on 2024-09-13.

Cite this Page

L-Cystathionine. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/cystathionine (retrieved 2024-11-09) (BioDeep RN: BioDeep_00000001337). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: C(CSCC(C(=O)O)N)C(C(=O)O)N
InChI: InChI=1S/C7H14N2O4S/c8-4(6(10)11)1-2-14-3-5(9)7(12)13/h4-5H,1-3,8-9H2,(H,10,11)(H,12,13)

描述信息

Cystathionine is a dipeptide formed by serine and homocysteine. Cystathioninuria is a prominent manifestation of vitamin-B6 deficiency. The transsulfuration of methionine yields homocysteine, which combines with serine to form cystathionine, the proximate precursor of cysteine through the enzymatic activity of cystathionase. In conditions in which cystathionine gamma-synthase or cystathionase is deficient, for example, there is cystathioninuria. Although cystathionine has not been detected in normal human serum or plasma by most conventional methods, gas chromatographic/mass spectrometric methodology detected a mean concentration of cystathionine in normal human serum of 140 nM, with a range of 65 to 301 nM. Cystathionine concentrations in CSF have been 10, 1, and 0.5 uM, and "not detected". Only traces (i.e., <1 uM) of cystathionine are present in normal CSF.587. Gamma-cystathionase deficiency (also known as Cystathioninuria), which is an autosomal recessive disorder (NIH: 2428), provided the first instance in which, in a human, the major biochemical abnormality due to a defined enzyme defect was clearly shown to be alleviated by administration of large doses of pyridoxine. The response in gamma-cystathionase-deficient patients is not attributable to correction of a preexisting deficiency of this vitamin (OMMBID, Chap. 88).
Isolated from Phallus impudicus (common stinkhorn)
CONFIDENCE standard compound; INTERNAL_ID 146
KEIO_ID C019; [MS2] KO008910
KEIO_ID C047
KEIO_ID C019
Acquisition and generation of the data is financially supported in part by CREST/JST.
CONFIDENCE standard compound; ML_ID 30
L-Cystathionine is a nonprotein thioether and is a key amino acid associated with the metabolic state of sulfur-containing amino acids. L-Cystathionine protects against Homocysteine-induced mitochondria-dependent apoptosis of vascular endothelial cells (HUVECs). L-Cystathionine plays an important role in cardiovascular protection[1][2].
L-Cystathionine is a nonprotein thioether and is a key amino acid associated with the metabolic state of sulfur-containing amino acids. L-Cystathionine protects against Homocysteine-induced mitochondria-dependent apoptosis of vascular endothelial cells (HUVECs). L-Cystathionine plays an important role in cardiovascular protection[1][2].

同义名列表

23 个代谢物同义名

(2S)-2-amino-4-{[(2R)-2-amino-2-carboxyethyl]sulfanyl}butanoic acid; [R-(R*,s*)]-2-amino-4-[(2-amino-2-carboxyethyl)thio]-butanoic acid; (2S)-2-amino-4-[(2R)-2-amino-2-carboxyethyl]sulfanylbutanoic acid; [R-(R*,s*)]-2-amino-4-[(2-amino-2-carboxyethyl)thio]-butanoate; S-[(2R)-2-Amino-2-carboxyethyl]-L-homocysteine; (R)-S-(2-Amino-2-carboxyethyl)-L-homocysteine; S-(beta-Amino-beta-carboxyethyl)homocysteine; S-(Β-amino-β-carboxyethyl)homocysteine; S-(b-Amino-b-carboxyethyl)homocysteine; L-(+)-Cystathionine; L(+)-Cystathionine; L-Cystathionine; L-Cystathione; Cystathionine; α-amino-γ-(2-amino-2-carboxyethylmercapto)-Butyric acid; 2-amino-4-(2-amino-2-carboxyethyl)sulfanylbutanoic acid; a-Amino-g-(2-amino-2-carboxyethylmercapto)-butyric acid; DL-S-(2-Amino-2-carboxyethyl)-homocysteine; Homocysteine,S-(2-amino-2-carboxyethyl)-; S-(2-Amino-2-carboxyethyl)homocysteine; DL,DL-allo-Cystathionine; DL-Allocystathionine; Cystathionine



数据库引用编号

56 个数据库交叉引用编号

分类词条

相关代谢途径

Reactome(6)

BioCyc(6)

PlantCyc(0)

代谢反应

412 个相关的代谢反应过程信息。

Reactome(71)

BioCyc(9)

WikiPathways(6)

Plant Reactome(324)

INOH(2)

PlantCyc(0)

COVID-19 Disease Map(0)

PathBank(0)

PharmGKB(0)

3 个相关的物种来源信息

在这里通过桑基图来展示出与当前的这个代谢物在我们的BioDeep知识库中具有相关联信息的其他代谢物。在这里进行关联的信息来源主要有:

  • PubMed: 来源于PubMed文献库中的文献信息,我们通过自然语言数据挖掘得到的在同一篇文献中被同时提及的相关代谢物列表,这个列表按照代谢物同时出现的文献数量降序排序,取前10个代谢物作为相关研究中关联性很高的代谢物集合展示在桑基图中。
  • NCBI Taxonomy: 通过文献数据挖掘,得到的代谢物物种来源信息关联。这个关联信息同样按照出现的次数降序排序,取前10个代谢物作为高关联度的代谢物集合展示在桑吉图上。
  • Chemical Taxonomy: 在物质分类上处于同一个分类集合中的其他代谢物
  • Chemical Reaction: 在化学反应过程中,存在为当前代谢物相关联的生化反应过程中的反应底物或者反应产物的关联代谢物信息。

点击图上的相关代谢物的名称,可以跳转到相关代谢物的信息页面。



文献列表

  • Alan Cunningham, Lieve Oudejans, Marjan Geugien, Diego A Pereira-Martins, Albertus Tj Wierenga, Ayşegül Erdem, Dominique Sternadt, Gerwin A Huls, Jan Jacob Schuringa. The 'non-essential' amino acid cysteine is required to prevent ferroptosis in acute myeloid leukemia. Blood advances. 2023 Oct; ?(?):. doi: 10.1182/bloodadvances.2023010786. [PMID: 37906522]
  • Rafael Rosell, Anisha Jain, Jordi Codony-Servat, Eloisa Jantus-Lewintre, Blake Morrison, Jordi Barretina Ginesta, María González-Cao. Biological insights in non-small cell lung cancer. Cancer biology & medicine. 2023 Jun; ?(?):. doi: 10.20892/j.issn.2095-3941.2023.0108. [PMID: 37381723]
  • Elena C Tore, Simone J P M Eussen, Nasser E Bastani, Pieter C Dagnelie, Amany K Elshorbagy, Pol Grootswagers, Viktor Kožich, Thomas Olsen, Helga Refsum, Kjetil Retterstøl, Coen DA Stehouwer, Emma T K Stolt, Kathrine J Vinknes, Marleen M J van Greevenbroek. The Associations of Habitual Intake of Sulfur Amino Acids, Proteins and Diet Quality with Plasma Sulfur Amino Acid Concentrations: The Maastricht Study. The Journal of nutrition. 2023 May; ?(?):. doi: 10.1016/j.tjnut.2023.05.008. [PMID: 37164267]
  • Chau M Tran, Shoko Mihara, Keisuke Yoshida, Toru Hisabori. Cystathionine-β-synthase X proteins negatively regulate NADPH-thioredoxin reductase C activity. Biochemical and biophysical research communications. 2023 04; 653(?):47-52. doi: 10.1016/j.bbrc.2023.02.055. [PMID: 36857899]
  • Fei Duan, Man-Man Li, Wei-Xia Li, Jin-Fa Tang, Zhi-Wan Wang. [Serum metabolomics of chronic obstructive pulmonary disease with lung-Qi deficiency syndrome]. Zhongguo Zhong yao za zhi = Zhongguo zhongyao zazhi = China journal of Chinese materia medica. 2022 Apr; 47(8):2251-2256. doi: 10.19540/j.cnki.cjcmm.20211203.501. [PMID: 35531742]
  • N Mota-Martorell, M Jové, R Berdún, R Pamplona. Plasma methionine metabolic profile is associated with longevity in mammals. Communications biology. 2021 06; 4(1):725. doi: 10.1038/s42003-021-02254-3. [PMID: 34117367]
  • Ferran Comas, Jèssica Latorre, Francisco Ortega, Núria Oliveras-Cañellas, Aina Lluch, Wifredo Ricart, José Manuel Fernández-Real, José María Moreno-Navarrete. Permanent cystathionine-β-Synthase gene knockdown promotes inflammation and oxidative stress in immortalized human adipose-derived mesenchymal stem cells, enhancing their adipogenic capacity. Redox biology. 2021 06; 42(?):101668. doi: 10.1016/j.redox.2020.101668. [PMID: 32800520]
  • Ingrid Kvestad, Adrian McCann, Ram K Chandyo, Lasse M Giil, Merina Shrestha, Manjeswori Ulak, Mari Hysing, Per M Ueland, Tor A Strand. One-Carbon Metabolism in Nepalese Infant-Mother Pairs and Child Cognition at 5 Years Old. The Journal of nutrition. 2021 04; 151(4):883-891. doi: 10.1093/jn/nxaa403. [PMID: 33484134]
  • Laurent Ouerdane, Eszter Borbála Both, Jiqian Xiang, Hongqing Yin, Yu Kang, Shuxun Shao, Katalin Kiszelák, Zsuzsa Jókai, Mihály Dernovics. Water soluble selenometabolome of Cardamine violifolia. Metallomics : integrated biometal science. 2020 12; 12(12):2032-2048. doi: 10.1039/d0mt00216j. [PMID: 33165451]
  • Mads M Svenningsson, Gard F T Svingen, Vegard Lysne, Per M Ueland, Grethe S Tell, Eva R Pedersen, Indu Dhar, Dennis W Nilsen, Ottar Nygård. Transsulfuration metabolites and the association with incident atrial fibrillation - An observational cohort study among Norwegian patients with stable angina pectoris. International journal of cardiology. 2020 Oct; 317(?):75-80. doi: 10.1016/j.ijcard.2020.05.010. [PMID: 32389762]
  • Yasuyuki Matoba, Masafumi Noda, Tomoki Yoshida, Kosuke Oda, Yuka Ezumi, Chiaki Yasutake, Hisae Izuhara-Kihara, Narandarai Danshiitsoodol, Takanori Kumagai, Masanori Sugiyama. Catalytic specificity of the Lactobacillus plantarum cystathionine γ-lyase presumed by the crystallographic analysis. Scientific reports. 2020 09; 10(1):14886. doi: 10.1038/s41598-020-71756-7. [PMID: 32913258]
  • X M Yuan, B Zhuan, P Li, X Zhao, T Wang, Z Yang. [Expression of nicotinamide adenine dinucleotide phosphate-reduced oxidase-4/reactive oxygen species and cystathionine-γ-lyase/hydrogen sulfide in patients with chronic obstructive pulmonary disease-related pulmonary hypertension]. Zhonghua nei ke za zhi. 2019 Oct; 58(10):770-776. doi: 10.3760/cma.j.issn.0578-1426.2019.10.009. [PMID: 31594176]
  • Laura Kasak, Constantina Bakolitsa, Zhiqiang Hu, Changhua Yu, Jasper Rine, Dago F Dimster-Denk, Gaurav Pandey, Greet De Baets, Yana Bromberg, Chen Cao, Emidio Capriotti, Rita Casadio, Joost Van Durme, Manuel Giollo, Rachel Karchin, Panagiotis Katsonis, Emanuela Leonardi, Olivier Lichtarge, Pier Luigi Martelli, David Masica, Sean D Mooney, Ayodeji Olatubosun, Predrag Radivojac, Frederic Rousseau, Lipika R Pal, Castrense Savojardo, Joost Schymkowitz, Janita Thusberg, Silvio C E Tosatto, Mauno Vihinen, Jouni Väliaho, Susanna Repo, John Moult, Steven E Brenner, Iddo Friedberg. Assessing computational predictions of the phenotypic effect of cystathionine-beta-synthase variants. Human mutation. 2019 09; 40(9):1530-1545. doi: 10.1002/humu.23868. [PMID: 31301157]
  • Maria F Mujica-Coopman, Dayana R Farias, Ana B Franco-Sena, Juliana S Vaz, Gilberto Kac, Yvonne Lamers. Maternal Plasma Pyridoxal 5'-Phosphate Concentration Is Inversely Associated with Plasma Cystathionine Concentration across All Trimesters in Healthy Pregnant Women. The Journal of nutrition. 2019 08; 149(8):1354-1362. doi: 10.1093/jn/nxz082. [PMID: 31098628]
  • Antonio Belardo, Federica Gevi, Lello Zolla. The concomitant lower concentrations of vitamins B6, B9 and B12 may cause methylation deficiency in autistic children. The Journal of nutritional biochemistry. 2019 08; 70(?):38-46. doi: 10.1016/j.jnutbio.2019.04.004. [PMID: 31151052]
  • Indu Dhar, Vegard Lysne, Gard F T Svingen, Per M Ueland, Jesse F Gregory, Kaare H Bønaa, Ottar K Nygård. Elevated plasma cystathionine is associated with increased risk of mortality among patients with suspected or established coronary heart disease. The American journal of clinical nutrition. 2019 06; 109(6):1546-1554. doi: 10.1093/ajcn/nqy391. [PMID: 31005968]
  • Lili Shi, Chao Han, Shuai Liu, Yiqun Liu, Xuesong Xiang. [Effects of methionine on the activity of cystathionine-β-synthase]. Wei sheng yan jiu = Journal of hygiene research. 2019 May; 48(3):463-467. doi: . [PMID: 31133135]
  • Thomas Olsen, Bente Øvrebø, Cheryl Turner, Nasser E Bastani, Helga Refsum, Kathrine J Vinknes. Combining Dietary Sulfur Amino Acid Restriction with Polyunsaturated Fatty Acid Intake in Humans: A Randomized Controlled Pilot Trial. Nutrients. 2018 Nov; 10(12):. doi: 10.3390/nu10121822. [PMID: 30477080]
  • Indu Dhar, Gard F T Svingen, Per M Ueland, Vegard Lysne, Mads M Svenningsson, Grethe S Tell, Ottar K Nygård. Plasma Cystathionine and Risk of Incident Stroke in Patients With Suspected Stable Angina Pectoris. Journal of the American Heart Association. 2018 09; 7(17):e008824. doi: 10.1161/jaha.118.008824. [PMID: 30371177]
  • Indu Dhar, Gard F T Svingen, Eva R Pedersen, Barbara DeRatt, Arve Ulvik, Elin Strand, Per M Ueland, Kaare H Bønaa, Jesse F Gregory, Ottar K Nygård. Plasma cystathionine and risk of acute myocardial infarction among patients with coronary heart disease: Results from two independent cohorts. International journal of cardiology. 2018 Sep; 266(?):24-30. doi: 10.1016/j.ijcard.2018.04.083. [PMID: 29728335]
  • Shozo Tomonaga, Hirofumi Okuyama, Tetsuya Tachibana, Ryosuke Makino. Effects of high ambient temperature on plasma metabolomic profiles in chicks. Animal science journal = Nihon chikusan Gakkaiho. 2018 Feb; 89(2):448-455. doi: 10.1111/asj.12951. [PMID: 29154397]
  • Barbara N DeRatt, Maria A Ralat, Vegard Lysne, Fariba Tayyari, Indu Dhar, Arthur S Edison, Timothy J Garrett, Øivind Midttun, Per Magne Ueland, Ottar Kjell Nygård, Jesse F Gregory. Metabolomic Evaluation of the Consequences of Plasma Cystathionine Elevation in Adults with Stable Angina Pectoris. The Journal of nutrition. 2017 09; 147(9):1658-1668. doi: 10.3945/jn.117.254029. [PMID: 28794210]
  • Yasuyuki Matoba, Tomoki Yoshida, Hisae Izuhara-Kihara, Masafumi Noda, Masanori Sugiyama. Crystallographic and mutational analyses of cystathionine β-synthase in the H2 S-synthetic gene cluster in Lactobacillus plantarum. Protein science : a publication of the Protein Society. 2017 04; 26(4):763-783. doi: 10.1002/pro.3123. [PMID: 28127810]
  • Wupeng Yan, Everett Stone, Yan Jessie Zhang. Structural Snapshots of an Engineered Cystathionine-γ-lyase Reveal the Critical Role of Electrostatic Interactions in the Active Site. Biochemistry. 2017 02; 56(6):876-885. doi: 10.1021/acs.biochem.6b01172. [PMID: 28106980]
  • Angelo D'Alessandro, Travis Nemkov, Lori K Bogren, Sandra L Martin, Kirk C Hansen. Comfortably Numb and Back: Plasma Metabolomics Reveals Biochemical Adaptations in the Hibernating 13-Lined Ground Squirrel. Journal of proteome research. 2017 02; 16(2):958-969. doi: 10.1021/acs.jproteome.6b00884. [PMID: 27991798]
  • Han Li, Mu Wang, Qiande Liang, Shuna Jin, Xiaojie Sun, Yangqian Jiang, Xingyun Pan, Yanqiu Zhou, Yang Peng, Bin Zhang, Aifen Zhou, Yiming Zhang, Zhong Chen, Jiangxia Cao, Hongling Zhang, Wei Xia, Tongzhang Zheng, Zongwei Cai, Yuanyuan Li, Shunqing Xu. Urinary metabolomics revealed arsenic exposure related to metabolic alterations in general Chinese pregnant women. Journal of chromatography. A. 2017 Jan; 1479(?):145-152. doi: 10.1016/j.chroma.2016.12.007. [PMID: 27988079]
  • Zheng Zhou, Mario Vailati-Riboni, Daniel N Luchini, Juan J Loor. Methionine and Choline Supply during the Periparturient Period Alter Plasma Amino Acid and One-Carbon Metabolism Profiles to Various Extents: Potential Role in Hepatic Metabolism and Antioxidant Status. Nutrients. 2016 Dec; 9(1):. doi: 10.3390/nu9010010. [PMID: 28036059]
  • Mélanie Pouget, Marie Perrot, Philippe Denis, Jacques Vuichoud, Dominique Dardevet, Karine Vidal, Denis Breuillé, Isabelle Papet. Long-term dietary supplementation with cystathionine improves tissue glutathione in ageing rats. Aging clinical and experimental research. 2016 Aug; 28(4):781-5. doi: 10.1007/s40520-015-0465-6. [PMID: 26514972]
  • Viktor Kožich, Jakub Krijt, Jitka Sokolová, Petra Melenovská, Pavel Ješina, Roman Vozdek, Tomáš Majtán, Jan P Kraus. Thioethers as markers of hydrogen sulfide production in homocystinurias. Biochimie. 2016 Jul; 126(?):14-20. doi: 10.1016/j.biochi.2016.01.001. [PMID: 26791043]
  • Clemens C Stoffregen, Elisabeth A Odin, Göran U Carlsson, Göran K Kurlberg, Hillevi G Björkqvist, Maria T Tångefjord, Bengt G Gustavsson. Reduced folate and serum vitamin metabolites in patients with rectal carcinoma: an open-label feasibility study of pemetrexed with folic acid and vitamin B12 supplementation. Anti-cancer drugs. 2016 Jun; 27(5):439-46. doi: 10.1097/cad.0000000000000345. [PMID: 26825869]
  • Rima Obeid, Susanne H Kirsch, Sarah Dilmann, Cosima Klein, Rudolf Eckert, Jürgen Geisel, Wolfgang Herrmann. Folic acid causes higher prevalence of detectable unmetabolized folic acid in serum than B-complex: a randomized trial. European journal of nutrition. 2016 Apr; 55(3):1021-8. doi: 10.1007/s00394-015-0916-z. [PMID: 25943647]
  • Lei Yin, Kevin Shengyang Yu, Kun Lu, Xiaozhong Yu. Benzyl butyl phthalate promotes adipogenesis in 3T3-L1 preadipocytes: A High Content Cellomics and metabolomic analysis. Toxicology in vitro : an international journal published in association with BIBRA. 2016 Apr; 32(?):297-309. doi: 10.1016/j.tiv.2016.01.010. [PMID: 26820058]
  • Kim M Holwerda, M Susanne Weedon-Fekjær, Anne C Staff, Ilja M Nolte, Harry van Goor, A Titia Lely, Marijke M Faas. The association of single nucleotide polymorphisms of the maternal cystathionine-β-synthase gene with early-onset preeclampsia. Pregnancy hypertension. 2016 Jan; 6(1):60-5. doi: 10.1016/j.preghy.2015.12.001. [PMID: 26955774]
  • Shyamchand Mayengbam, Sara Raposo, Michel Aliani, James D House. A Vitamin B-6 Antagonist from Flaxseed Perturbs Amino Acid Metabolism in Moderately Vitamin B-6-Deficient Male Rats. The Journal of nutrition. 2016 Jan; 146(1):14-20. doi: 10.3945/jn.115.219378. [PMID: 26581680]
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