Cysteine S-sulfate (BioDeep_00000001364)

 

Secondary id: BioDeep_00000400345, BioDeep_00000402816, BioDeep_00000405350

natural product human metabolite PANOMIX_OTCML-2023 Endogenous blood metabolite


代谢物信息卡片


(2R)-2-amino-3-(sulfosulfanyl)propanoic acid

化学式: C3H7NO5S2 (200.97656519999998)
中文名称: S-磺基-L-半胱氨酸
谱图信息: 最多检出来源 Homo sapiens(feces) 0.97%

Reviewed

Last reviewed on 2024-09-13.

Cite this Page

Cysteine S-sulfate. BioDeep Database v3. PANOMIX ltd, a top metabolomics service provider from China. https://query.biodeep.cn/s/cysteine_s-sulfate (retrieved 2024-11-25) (BioDeep RN: BioDeep_00000001364). Licensed under the Attribution-Noncommercial 4.0 International License (CC BY-NC 4.0).

分子结构信息

SMILES: C(C(C(=O)O)N)SS(=O)(=O)O
InChI: InChI=1S/C3H7NO5S2/c4-2(3(5)6)1-10-11(7,8)9/h2H,1,4H2,(H,5,6)(H,7,8,9)/t2-/m0/s1

描述信息

Cysteine-S-sulfate (SSC) is produced by reaction of inorganic sulfite and cystine by a yet unknown pathway and is a very potent NMDA-receptor agonist. Electrophysiological studies have shown that SSC displays depolarizing properties similar to glutamate. Patients affected with either Molybdenum cofactor deficiency (MOCOD, an autosomal recessive disease that leads to a combined deficiency of the enzymes sulphite oxidase, an enzyme that catalyzes the conversion of sulfite to inorganic sulfate, xanthine dehydrogenase and aldehyde oxidase) or isolated sulphite oxidase deficiency (ISOD, an extremely rare autosomal recessive disorder with identical clinical manifestations to MOCOD) excrete elevated levels of SSC. This rare disorder is associated with brain damage (seizures, spastic quadriplegia, and cerebral atrophy), mental retardation, dislocated ocular lenses, blindness, and excretion in the urine of abnormally large amounts of SSC, sulfite, and thiosulfate but no inorganic sulfate (PMID: 17764028, 15558695).
Acquisition and generation of the data is financially supported in part by CREST/JST.
KEIO_ID C127; [MS2] KO008902
KEIO_ID C127

同义名列表

36 个代谢物同义名

(2R)-2-amino-3-(sulfosulfanyl)propanoic acid; L-Cysteine hydrogen sulphate (ester); L-Cysteine hydrogen sulfate (ester); L-Cysteine hydrogen sulphuric acid; S-Sulphocysteine, monosodium salt; L-Cysteine hydrogen sulfuric acid; L-Cysteine hydrogen sulphate; L-Cysteine S-sulphuric acid; L-Cysteine hydrogen sulfate; L-Cysteine S-sulfuric acid; Cysteinyl-S-sulphonic acid; Cysteine-S-sulphuric acid; Cysteinyl-S-sulfonic acid; S-Sulphocysteine ion (1-); Cysteine-S-sulphonic acid; Cysteine-S-sulfuric acid; Cysteine-S-sulfonic acid; Alaninethiosulfuric acid; S-Cysteinesulfonic acid; L-Cysteinesulfonic acid; Cysteinyl-S-sulphonate; L-Cysteine-S-sulfonate; L-Cysteine S-sulphate; Cysteinyl-S-sulfonate; Cysteine-S-sulphonate; Cysteine-S-sulfonate; L-Cysteine S-sulfate; Cystein-S-sulfosaure; Cysteine-S-sulphate; S-SulphO-L-cysteine; S-sulfo-L-cysteine; Cysteine S-sulfate; Cysteine-S-sulfate; S-Sulphocysteine; S-Sulfocysteine; S-Sulfo-L-cysteine



数据库引用编号

40 个数据库交叉引用编号

分类词条

相关代谢途径

Reactome(0)

BioCyc(0)

PlantCyc(0)

代谢反应

3 个相关的代谢反应过程信息。

Reactome(0)

BioCyc(0)

WikiPathways(1)

Plant Reactome(0)

INOH(0)

PlantCyc(0)

COVID-19 Disease Map(0)

PathBank(2)

PharmGKB(0)

22 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

  • Yi Jiang, Brandon Mistretta, Sarah H Elsea, Qin Sun. Development of a rapid UPLC-MS/MS determination of urine sulfocysteine for diagnosis of sulfocysteinuria and molybdenum co-factor deficiencies. Bioanalysis. 2018 May; 10(10):747-756. doi: 10.4155/bio-2017-0278. [PMID: 29774761]
  • Avadh Kumar, Borislav Dejanovic, Florian Hetsch, Marcus Semtner, Debora Fusca, Sita Arjune, Jose Angel Santamaria-Araujo, Aline Winkelmann, Scott Ayton, Ashley I Bush, Peter Kloppenburg, Jochen C Meier, Guenter Schwarz, Abdel Ali Belaidi. S-sulfocysteine/NMDA receptor-dependent signaling underlies neurodegeneration in molybdenum cofactor deficiency. The Journal of clinical investigation. 2017 12; 127(12):4365-4378. doi: 10.1172/jci89885. [PMID: 29106383]
  • Joanna Jakubiczka-Smorag, Jose Angel Santamaria-Araujo, Imke Metz, Avadh Kumar, Samy Hakroush, Wolfgang Brueck, Guenter Schwarz, Peter Burfeind, Jochen Reiss, Lukasz Smorag. Mouse model for molybdenum cofactor deficiency type B recapitulates the phenotype observed in molybdenum cofactor deficient patients. Human genetics. 2016 07; 135(7):813-26. doi: 10.1007/s00439-016-1676-4. [PMID: 27138983]
  • H Westerlinck, L Meylaerts, M R Van Hoestenberghe, A Rossi. Sulfite oxidase deficiency in a newborn. JBR-BTR : organe de la Societe royale belge de radiologie (SRBR) = orgaan van de Koninklijke Belgische Vereniging voor Radiologie (KBVR). 2014 Mar; 97(2):113-4. doi: 10.5334/jbr-btr.40. [PMID: 25073245]
  • Cecilia Gotor, Luis C Romero. S-sulfocysteine synthase function in sensing chloroplast redox status. Plant signaling & behavior. 2013 Mar; 8(3):e23313. doi: 10.4161/psb.23313. [PMID: 23333972]
  • Abdel Ali Belaidi, Guenter Schwarz. Molybdenum cofactor deficiency: metabolic link between taurine and S-sulfocysteine. Advances in experimental medicine and biology. 2013; 776(?):13-9. doi: 10.1007/978-1-4614-6093-0_2. [PMID: 23392866]
  • Philippa B Mills, Emma J Footitt, Serkan Ceyhan, Paula J Waters, Cornelis Jakobs, Peter T Clayton, Eduard A Struys. Urinary AASA excretion is elevated in patients with molybdenum cofactor deficiency and isolated sulphite oxidase deficiency. Journal of inherited metabolic disease. 2012 Nov; 35(6):1031-6. doi: 10.1007/s10545-012-9466-1. [PMID: 22403017]
  • Hankuil Yi, Joseph M Jez. Assessing functional diversity in the soybean β-substituted alanine synthase enzyme family. Phytochemistry. 2012 Nov; 83(?):15-24. doi: 10.1016/j.phytochem.2012.08.003. [PMID: 22986002]
  • Marek Bajda, Kamil J Kuder, Dorota Lażewska, Katarzyna Kieć-Kononowicz, Anna Więckowska, Michalina Ignasik, Natalia Guzior, Jakub Jończyk, Barbara Malawska. Dual-acting diether derivatives of piperidine and homopiperidine with histamine H(3) receptor antagonistic and anticholinesterase activity. Archiv der Pharmazie. 2012 Aug; 345(8):591-7. doi: 10.1002/ardp.201200018. [PMID: 22549828]
  • Kenjiro Kikuchi, Shin-ichiro Hamano, Hiroshi Mochizuki, Kimiyoshi Ichida, Hiroyuki Ida. Molybdenum cofactor deficiency mimics cerebral palsy: differentiating factors for diagnosis. Pediatric neurology. 2012 Aug; 47(2):147-9. doi: 10.1016/j.pediatrneurol.2012.04.013. [PMID: 22759696]
  • Hongqing Li, Yongmin Zhang, Sylvain Favre, Pierre Vogel, Matthieu Sollogoub, Yves Blériot. Synthesis of branched seven-membered 1-N-iminosugars and their evaluation as glycosidase inhibitors. Carbohydrate research. 2012 Jul; 356(?):110-4. doi: 10.1016/j.carres.2011.10.039. [PMID: 22129839]
  • Kuo-Ching Wang, Ching-Hua Kuo, Tze-Feng Tian, Mong-Hsun Tsai, Yin-Mei Chiung, Chun-Ming Hsiech, Sung-Jeng Tsai, San-Yuan Wang, Dong-Ming Tsai, Chiang-Ching Huang, Y Jane Tseng. Metabolomic characterization of laborers exposed to welding fumes. Chemical research in toxicology. 2012 Mar; 25(3):676-86. doi: 10.1021/tx200465e. [PMID: 22292500]
  • Emma J Footitt, Simon J Heales, Philippa B Mills, George F G Allen, Marcus Oppenheim, Peter T Clayton. Pyridoxal 5'-phosphate in cerebrospinal fluid; factors affecting concentration. Journal of inherited metabolic disease. 2011 Apr; 34(2):529-38. doi: 10.1007/s10545-011-9279-7. [PMID: 21305354]
  • Jeffrey G Varnes, Janet M Forst, Tiffany N Hoerter, Christopher R Holmquist, Deidre E Wilkins, Gaochao Tian, Gerald Jonak, Xia Wang, William M Potts, Michael W Wood, Cristóbal Alhambra, Todd A Brugel, Jeffrey S Albert. Identification of N-(2-(azepan-1-yl)-2-phenylethyl)-benzenesulfonamides as novel inhibitors of GlyT1. Bioorganic & medicinal chemistry letters. 2010 Aug; 20(16):4878-81. doi: 10.1016/j.bmcl.2010.06.085. [PMID: 20637614]
  • Maria Angeles Bermúdez, Maria Angeles Páez-Ochoa, Cecilia Gotor, Luis C Romero. Arabidopsis S-sulfocysteine synthase activity is essential for chloroplast function and long-day light-dependent redox control. The Plant cell. 2010 Feb; 22(2):403-16. doi: 10.1105/tpc.109.071985. [PMID: 20179139]
  • Ingrid Brucknerova, Darina Behulova, Vladimir Bzduch, Mojmir Mach, Michal Dubovicky, Eduard Ujhazy. Newborn with neonatal form of molybdenum cofactor deficiency - the first patient in the Slovak Republic. Neuro endocrinology letters. 2010; 31 Suppl 2(?):5-7. doi: . [PMID: 21187823]
  • L H Ngu, B Afroze, B C Chen, O Affandi, M Y Zabedah. Molybdenum cofactor deficiency in a Malaysian child. Singapore medical journal. 2009 Oct; 50(10):e365-7. doi: . [PMID: 19907877]
  • Sorin J Brull, Mohamed Naguib. Selective reversal of muscle relaxation in general anesthesia: focus on sugammadex. Drug design, development and therapy. 2009 Sep; 3(?):119-29. doi: 10.2147/dddt.s3868. [PMID: 19920928]
  • Luigi Gennari, Daniela Merlotti, Vincenzo De Paola, Giuseppe Martini, Ranuccio Nuti. Bazedoxifene for the prevention of postmenopausal osteoporosis. Therapeutics and clinical risk management. 2008 Dec; 4(6):1229-42. doi: 10.2147/tcrm.s3476. [PMID: 19337430]
  • A Macaya, L Brunso, N Fernández-Castillo, J A Arranz, H B Ginjaar, E Cuenca-León, R Corominas, M Roig, B Cormand. Molybdenum cofactor deficiency presenting as neonatal hyperekplexia: a clinical, biochemical and genetic study. Neuropediatrics. 2005 Dec; 36(6):389-94. doi: 10.1055/s-2005-872877. [PMID: 16429380]
  • Emma E Hobson, Sumesh Thomas, Patricia M Crofton, Alison D Murray, John C S Dean, David Lloyd. Isolated sulphite oxidase deficiency mimics the features of hypoxic ischaemic encephalopathy. European journal of pediatrics. 2005 Nov; 164(11):655-9. doi: 10.1007/s00431-005-1729-5. [PMID: 16025295]
  • Wen-Hann Tan, Florian S Eichler, Sadaf Hoda, Melissa S Lee, Hagit Baris, Catherine A Hanley, P Ellen Grant, Kalpathy S Krishnamoorthy, Vivian E Shih. Isolated sulfite oxidase deficiency: a case report with a novel mutation and review of the literature. Pediatrics. 2005 Sep; 116(3):757-66. doi: 10.1542/peds.2004-1897. [PMID: 16140720]
  • M Z Seidahmed, E A Alyamani, M S Rashed, A A Saadallah, O B Abdelbasit, M M Shaheed, A Rasheed, F A Hamid, M A Sabry. Total truncation of the molybdopterin/dimerization domains of SUOX protein in an Arab family with isolated sulfite oxidase deficiency. American journal of medical genetics. Part A. 2005 Jul; 136(2):205-9. doi: 10.1002/ajmg.a.30796. [PMID: 15952210]
  • Mohamed S Rashed, Amal A A Saadallah, Zuhair Rahbeeni, Wafaa Eyaid, Mohamed Z Seidahmed, Saad Al-Shahwan, Mustafa A M Salih, Mohammad E Osman, Mohamed Al-Amoudi, Lujane Al-Ahaidib, Minnie Jacob. Determination of urinary S-sulphocysteine, xanthine and hypoxanthine by liquid chromatography-electrospray tandem mass spectrometry. Biomedical chromatography : BMC. 2005 Apr; 19(3):223-30. doi: 10.1002/bmc.439. [PMID: 15558695]
  • Toyofumi Nakanishi, Takako Sato, Saburo Sakoda, Masanori Yoshioka, Akira Shimizu. Modification of cysteine residue in transthyretin and a synthetic peptide: analyses by electrospray ionization mass spectrometry. Biochimica et biophysica acta. 2004 Apr; 1698(1):45-53. doi: 10.1016/j.bbapap.2003.10.005. [PMID: 15063314]
  • Christine B Breitenlechner, Thomas Wegge, Laurent Berillon, Klaus Graul, Klaus Marzenell, Walter-Gunar Friebe, Ulrike Thomas, Ralf Schumacher, Robert Huber, Richard A Engh, Birgit Masjost. Structure-based optimization of novel azepane derivatives as PKB inhibitors. Journal of medicinal chemistry. 2004 Mar; 47(6):1375-90. doi: 10.1021/jm0310479. [PMID: 14998327]
  • H F Lee, B S C Mak, C S Chi, C R Tsai, C H Chen, S G Shu. A novel mutation in neonatal isolated sulphite oxidase deficiency. Neuropediatrics. 2002 Aug; 33(4):174-9. doi: 10.1055/s-2002-34491. [PMID: 12368985]
  • H S Chong, K Garmestani, L H Bryant, M W Brechbiel. Synthesis of DTPA analogues derived from piperidine and azepane: potential contrast enhancement agents for magnetic resonance imaging. The Journal of organic chemistry. 2001 Nov; 66(23):7745-50. doi: 10.1021/jo0106344. [PMID: 11701031]
  • M Topcu, T Coskun, G Haliloglu, I Saatci. Molybdenum cofactor deficiency: report of three cases presenting as hypoxic-ischemic encephalopathy. Journal of child neurology. 2001 Apr; 16(4):264-70. doi: 10.1177/088307380101600406. [PMID: 11332461]
  • J L Johnson, K V Rajagopalan. An HPLC assay for detection of elevated urinary S-sulphocysteine, a metabolic marker of sulphite oxidase deficiency. Journal of inherited metabolic disease. 1995; 18(1):40-7. doi: 10.1007/bf00711371. [PMID: 7623441]
  • G A Qureshi, S M Baig. Application of high performance liquid chromatography in study of sulphur amino acid metabolism in uremic patients. Biochemistry and molecular biology international. 1993 Feb; 29(2):359-68. doi: NULL. [PMID: 8495218]
  • H C Winter, T Ueda. Glutamate uptake system in the presynaptic vesicle: glutamic acid analogs as inhibitors and alternate substrates. Neurochemical research. 1993 Jan; 18(1):79-85. doi: 10.1007/bf00966925. [PMID: 8096630]
  • J Dunlop, A Grieve, I Damgaard, A Schousboe, R Griffiths. Sulphur-containing excitatory amino acid-evoked Ca(2+)-independent release of D-[3H]aspartate from cultured cerebellar granule cells: the role of glutamate receptor activation coupled to reversal of the acidic amino acid plasma membrane carrier. Neuroscience. 1992 Sep; 50(1):107-15. doi: 10.1016/0306-4522(92)90385-f. [PMID: 1357589]
  • P Kamoun, P Tardy. Therapeutic attempts in sulphite oxidase deficiency. European journal of pediatrics. 1990 May; 149(8):594. doi: 10.1007/bf01957702. [PMID: 2347359]
  • P Parvy, J Bardet, D Rabier, J P Bonnefont, P Kamoun. A new pitfall in plasma amino acid analysis. Clinical chemistry. 1989 Jan; 35(1):178. doi: 10.1093/clinchem/35.1.178. [PMID: 2910564]
  • P Tardy, P Parvy, C Charpentier, J P Bonnefont, J M Saudubray, P Kamoun. Attempt at therapy in sulphite oxidase deficiency. Journal of inherited metabolic disease. 1989; 12(1):94-5. doi: 10.1007/bf01805537. [PMID: 2501590]
  • C L Weinstein, O W Griffith. Cysteinesulfonate and beta-sulfopyruvate metabolism. Partitioning between decarboxylation, transamination, and reduction pathways. The Journal of biological chemistry. 1988 Mar; 263(8):3735-43. doi: . [PMID: 3346220]
  • C Vianey-Liaud, P Desjacques, J Gaulme, C Dorche, P Vanlieferinghen, P Dechelotte, P Divry. A new case of isolated sulphite oxidase deficiency with rapid fatal outcome. Journal of inherited metabolic disease. 1988; 11(4):425-6. doi: 10.1007/bf01800433. [PMID: 3149702]
  • W Moore, H L Wiener, A Meister. Inactivation of gamma-glutamylcysteine synthetase, but not of glutamine synthetase, by S-sulfocysteine and S-sulfohomocysteine. The Journal of biological chemistry. 1987 Dec; 262(35):16771-7. doi: . [PMID: 2890640]
  • NULL. Molybdenum deficiency in TPN. Nutrition reviews. 1987 Nov; 45(11):337-41. doi: 10.1111/j.1753-4887.1987.tb00986.x. [PMID: 3141849]
  • B Grego, I R Van Driel, J W Goding, E C Nice, R J Simpson. Use of microbore high-performance liquid chromatography for purifying subnanomole levels of polypeptides for microsequencing. Structural studies on the murine plasma cell antigen PC-1. International journal of peptide and protein research. 1986 Feb; 27(2):201-7. doi: 10.1111/j.1399-3011.1986.tb01811.x. [PMID: 3084398]
  • D A Keller, D B Menzel. Picomole analysis of glutathione, glutathione disulfide, glutathione S-sulfonate, and cysteine S-sulfonate by high-performance liquid chromatography. Analytical biochemistry. 1985 Dec; 151(2):418-23. doi: 10.1016/0003-2697(85)90197-6. [PMID: 4096380]
  • B Kågedal, M Källberg, B Sörbo. Determination of S-sulfocysteine in urine by high-performance liquid chromatography. Journal of chromatography. 1983 Sep; 276(2):418-22. doi: 10.1016/s0378-4347(00)85110-5. [PMID: 6630390]
  • G Cederblad. On the occurrence of cysteine sulfinate in human urine. Biochemical medicine. 1980 Oct; 24(2):103-9. doi: 10.1016/0006-2944(80)90001-0. [PMID: 7458919]
  • J D deBethizy, J C Street. A specific gas chromatographic analysis for cysteinyl-S-sulfonate residues in physiologically significant proteins treated with sulfite. Journal of analytical toxicology. 1980 Jul; 4(4):175-80. doi: 10.1093/jat/4.4.175. [PMID: 7464069]
  • J W Olney, C H Misra, T de Gubareff. Cysteine-S-sulfate: brain damaging metabolite in sulfite oxidase deficiency. Journal of neuropathology and experimental neurology. 1975 Mar; 34(2):167-77. doi: 10.1097/00005072-197503000-00005. [PMID: 1123650]
  • . . . . doi: . [PMID: 20699647]