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Chemical Compound Review

cystine     2-amino-3-(2-amino-2- carboxy...

Synonyms: Nephrin, cistina, Zystin, D-Cystine, DL-CYSTINE, ...
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Disease relevance of cystine


Psychiatry related information on cystine

  • Patient self-assessment of medical compliance was uniformly high regardless of physician perceptions or treatment results.CONCLUSIONS The durability of medically treating patients with cystinuria is limited with only a small percent able to achieve and maintain the goal of decreasing cystine below the saturation concentration [6].
  • Therefore, because of its high yield in the native disulfide arrangement and its high permissiveness for sequence mutation in the 4-7 loop, such a stabilized cystine-stabilized alpha-helical motif could be a valuable scaffold for the presentation of a library of constrained short peptides [7].
  • Prevention of cystine cross-links during this critical period of cuticle assembly by peptide-FMK inhibitors may represent a potential control mechanism having a novel mechanism of action [8].
  • In a similar group of cats fed 0.05% taurine in addition to an excess of cystine, four cats died after showing minimal symptoms of lethargy and unsteadiness and the remainder showed no adverse effects [9].
  • The addition of cystine to the regime of this child, without any other dietary modification, resulted in a complete remission [10].

High impact information on cystine

  • Nck links nephrin to actin in kidney podocytes [11].
  • Two papers, one in Nature (Jones et al., 2006) and the other in the Journal of Clinical Investigation (Verma et al., 2006) show that Nck adaptor proteins connect phosphorylated nephrin with actin polymerization in podocyte foot processes, structures important for slit-diaphragm formation in the kidney [11].
  • Furthermore, both Ero1p and Erv2p display essential dicysteine motifs on mobile polypeptide segments, suggesting that shuttling electrons to a rigid active site using a flexible strand is a fundamental feature of disulfide-generating flavoenzymes [12].
  • Cystinuria (MIM 220100) is a common recessive disorder of renal reabsorption of cystine and dibasic amino acids [13].
  • Images in clinical medicine. Cystine crystals in Fanconi's syndrome [14].

Chemical compound and disease context of cystine


Biological context of cystine


Anatomical context of cystine

  • Because high concentrations of ascorbic acid (0.57 mM) lower the free (nonprotein) cystine content of cultured cystinotic skin fibroblasts by over 50 per cent, we did a double-blind clinical trial to establish whether this drug would benefit cystinotic children [24].
  • In our study, the mean cystine depletion from leukocytes was 82 percent [25].
  • Certain continuous lymphoid and myeloid tumor cell lines of rodent origin are unable to grow in tissue culture in the absence of pre-formed L-cystine (CYS) [5].
  • Transport interaction of cystine and dibasic amino acids in renal brush border vesicles [26].
  • Supporting a role for CD2AP in the specialized cell junction known as the slit diaphragm, CD2AP associated with nephrin, the primary component of the slit diaphragm [27].

Associations of cystine with other chemical compounds


Gene context of cystine


Analytical, diagnostic and therapeutic context of cystine


  1. Successful pregnancy despite placental cystine crystals in a woman with nephropathic cystinosis. Reiss, R.E., Kuwabara, T., Smith, M.L., Gahl, W.A. N. Engl. J. Med. (1988) [Pubmed]
  2. Cystinuria caused by mutations in rBAT, a gene involved in the transport of cystine. Calonge, M.J., Gasparini, P., Chillarón, J., Chillón, M., Gallucci, M., Rousaud, F., Zelante, L., Testar, X., Dallapiccola, B., Di Silverio, F. Nat. Genet. (1994) [Pubmed]
  3. A novel gene encoding an integral membrane protein is mutated in nephropathic cystinosis. Town, M., Jean, G., Cherqui, S., Attard, M., Forestier, L., Whitmore, S.A., Callen, D.F., Gribouval, O., Broyer, M., Bates, G.P., van't Hoff, W., Antignac, C. Nat. Genet. (1998) [Pubmed]
  4. Myopathy and cystine storage in muscles in a patient with nephropathic cystinosis. Gahl, W.A., Dalakas, M.C., Charnas, L., Chen, K.T., Pezeshkpour, G.H., Kuwabara, T., Davis, S.L., Chesney, R.W., Fink, J., Hutchison, H.T. N. Engl. J. Med. (1988) [Pubmed]
  5. Accumulation of cystine auxotrophic thymocytes accompanying type C viral leukemogenesis in the mouse. Livingston, D.M., Ferguson, C., Gollogly, R., Lazarus, H. Cell (1976) [Pubmed]
  6. Durability of the medical management of cystinuria. Pietrow, P.K., Auge, B.K., Weizer, A.Z., Delvecchio, F.C., Silverstein, A.D., Mathias, B., Albala, D.M., Preminger, G.M. J. Urol. (2003) [Pubmed]
  7. The chimeric peptide [Lys(-2)-Arg(-1)]-sarafotoxin-S6b, composed of the endothelin pro-sequence and sarafotoxin, retains the salt-bridge staple between Arg(-1) and Asp8 previously observed in [Lys(-2)-Arg(-1)]-endothelin. Implications of this salt-bridge in the contractile activity and the oxidative folding reaction. Aumelas, A., Chiche, L., Kubo, S., Chino, N., Watanabe, T.X., Kobayashi, Y. Eur. J. Biochem. (1999) [Pubmed]
  8. Cuticular collagen synthesis by Ascaris suum during development from the third to fourth larval stage: identification of a potential chemotherapeutic agent with a novel mechanism of action. Rhoads, M.L., Fetterer, R.H., Urban, J.F. J. Parasitol. (2001) [Pubmed]
  9. Cystine neurotoxicity is increased by taurine deficiency. Sturman, J.A., Messing, J.M., Gargano, A.D., Rerecich, M., Imaki, H., Rudelli, R. Neurotoxicology (1989) [Pubmed]
  10. Cystine deficiency during dietotherapy of homocystinemia. Sansaricq, C., Garg, S., Norton, P.M., Phansalkar, S.V., Snyderman, S.E. Acta paediatrica Scandinavica. (1975) [Pubmed]
  11. Nck links nephrin to actin in kidney podocytes. Tryggvason, K., Pikkarainen, T., Patrakka, J. Cell (2006) [Pubmed]
  12. Structure of Ero1p, source of disulfide bonds for oxidative protein folding in the cell. Gross, E., Kastner, D.B., Kaiser, C.A., Fass, D. Cell (2004) [Pubmed]
  13. Non-type I cystinuria caused by mutations in SLC7A9, encoding a subunit (bo,+AT) of rBAT. Feliubadaló, L., Font, M., Purroy, J., Rousaud, F., Estivill, X., Nunes, V., Golomb, E., Centola, M., Aksentijevich, I., Kreiss, Y., Goldman, B., Pras, M., Kastner, D.L., Pras, E., Gasparini, P., Bisceglia, L., Beccia, E., Gallucci, M., de Sanctis, L., Ponzone, A., Rizzoni, G.F., Zelante, L., Bassi, M.T., George, A.L., Manzoni, M., De Grandi, A., Riboni, M., Endsley, J.K., Ballabio, A., Borsani, G., Reig, N., Fernández, E., Estévez, R., Pineda, M., Torrents, D., Camps, M., Lloberas, J., Zorzano, A., Palacín, M. Nat. Genet. (1999) [Pubmed]
  14. Images in clinical medicine. Cystine crystals in Fanconi's syndrome. Singh, J.K., Kusior, M.F. N. Engl. J. Med. (1999) [Pubmed]
  15. Effect of glutamine on cystine excretion in a patient with cystinuria. Miyagi, K., Nakada, F., Ohshiro, S. N. Engl. J. Med. (1979) [Pubmed]
  16. At the acidic edge: emerging functions for lysosomal membrane proteins. Eskelinen, E.L., Tanaka, Y., Saftig, P. Trends Cell Biol. (2003) [Pubmed]
  17. Glutamate toxicity in a neuronal cell line involves inhibition of cystine transport leading to oxidative stress. Murphy, T.H., Miyamoto, M., Sastre, A., Schnaar, R.L., Coyle, J.T. Neuron (1989) [Pubmed]
  18. Pantethine and cystamine deplete cystine from cystinotic fibroblasts via efflux of cysteamine-cysteine mixed disulfide. Butler, J.D., Zatz, M. J. Clin. Invest. (1984) [Pubmed]
  19. Plasma cysteine, cystine, and glutathione in cirrhosis. Chawla, R.K., Lewis, F.W., Kutner, M.H., Bate, D.M., Roy, R.G., Rudman, D. Gastroenterology (1984) [Pubmed]
  20. Effects of cysteamine therapy in nephropathic cystinosis. Yudkoff, M., Foreman, J.W., Segal, S. N. Engl. J. Med. (1981) [Pubmed]
  21. Positionally cloned gene for a novel glomerular protein--nephrin--is mutated in congenital nephrotic syndrome. Kestilä, M., Lenkkeri, U., Männikkö, M., Lamerdin, J., McCready, P., Putaala, H., Ruotsalainen, V., Morita, T., Nissinen, M., Herva, R., Kashtan, C.E., Peltonen, L., Holmberg, C., Olsen, A., Tryggvason, K. Mol. Cell (1998) [Pubmed]
  22. The cystine-knot growth-factor superfamily. Sun, P.D., Davies, D.R. Annual review of biophysics and biomolecular structure. (1995) [Pubmed]
  23. Mechanism of augmentation of the antibody response in vitro by 2-mercaptoethanol in murine lymphocytes. I. 2-Mercaptoethanol-induced stimulation of the uptake of cystine, an essential amino acid. Ohmori, H., Yamamoto, I. J. Exp. Med. (1982) [Pubmed]
  24. Ineffectiveness of ascorbic acid therapy in nephropathic cystinosis. Schneider, J.A., Schlesselman, J.J., Mendoza, S.A., Orloff, S., Thoene, J.G., Kroll, W.A., Godfrey, A.D., Schulman, J.D. N. Engl. J. Med. (1979) [Pubmed]
  25. Cysteamine therapy for children with nephropathic cystinosis. Gahl, W.A., Reed, G.F., Thoene, J.G., Schulman, J.D., Rizzo, W.B., Jonas, A.J., Denman, D.W., Schlesselman, J.J., Corden, B.J., Schneider, J.A. N. Engl. J. Med. (1987) [Pubmed]
  26. Transport interaction of cystine and dibasic amino acids in renal brush border vesicles. Segal, S., McNamara, P.D., Pepe, L.M. Science (1977) [Pubmed]
  27. Congenital nephrotic syndrome in mice lacking CD2-associated protein. Shih, N.Y., Li, J., Karpitskii, V., Nguyen, A., Dustin, M.L., Kanagawa, O., Miner, J.H., Shaw, A.S. Science (1999) [Pubmed]
  28. Swallowing dysfunction in nephropathic cystinosis. Sonies, B.C., Ekman, E.F., Andersson, H.C., Adamson, M.D., Kaler, S.G., Markello, T.C., Gahl, W.A. N. Engl. J. Med. (1990) [Pubmed]
  29. Thiol-mediated redox regulation of intestinal lamina propria T lymphocytes. Sido, B., Braunstein, J., Breitkreutz, R., Herfarth, C., Meuer, S.C. J. Exp. Med. (2000) [Pubmed]
  30. Induction of cystine transport activity in mouse peritoneal macrophages. Watanabe, H., Bannai, S. J. Exp. Med. (1987) [Pubmed]
  31. Neuroadaptations in cystine-glutamate exchange underlie cocaine relapse. Baker, D.A., McFarland, K., Lake, R.W., Shen, H., Tang, X.C., Toda, S., Kalivas, P.W. Nat. Neurosci. (2003) [Pubmed]
  32. Primary structures of three human neutrophil defensins. Selsted, M.E., Harwig, S.S., Ganz, T., Schilling, J.W., Lehrer, R.I. J. Clin. Invest. (1985) [Pubmed]
  33. HIV-1 Tat reduces nephrin in human podocytes: a potential mechanism for enhanced glomerular permeability in HIV-associated nephropathy. Doublier, S., Zennaro, C., Spatola, T., Lupia, E., Bottelli, A., Deregibus, M.C., Carraro, M., Conaldi, P.G., Camussi, G. AIDS (2007) [Pubmed]
  34. Podocin, a raft-associated component of the glomerular slit diaphragm, interacts with CD2AP and nephrin. Schwarz, K., Simons, M., Reiser, J., Saleem, M.A., Faul, C., Kriz, W., Shaw, A.S., Holzman, L.B., Mundel, P. J. Clin. Invest. (2001) [Pubmed]
  35. Transcriptional induction of slit diaphragm genes by Lmx1b is required in podocyte differentiation. Miner, J.H., Morello, R., Andrews, K.L., Li, C., Antignac, C., Shaw, A.S., Lee, B. J. Clin. Invest. (2002) [Pubmed]
  36. Altered podocyte structure in GLEPP1 (Ptpro)-deficient mice associated with hypertension and low glomerular filtration rate. Wharram, B.L., Goyal, M., Gillespie, P.J., Wiggins, J.E., Kershaw, D.B., Holzman, L.B., Dysko, R.C., Saunders, T.L., Samuelson, L.C., Wiggins, R.C. J. Clin. Invest. (2000) [Pubmed]
  37. Homer proteins regulate sensitivity to cocaine. Szumlinski, K.K., Dehoff, M.H., Kang, S.H., Frys, K.A., Lominac, K.D., Klugmann, M., Rohrer, J., Griffin, W., Toda, S., Champtiaux, N.P., Berry, T., Tu, J.C., Shealy, S.E., During, M.J., Middaugh, L.D., Worley, P.F., Kalivas, P.W. Neuron (2004) [Pubmed]
  38. Nephritogenic mAb 5-1-6 is directed at the extracellular domain of rat nephrin. Topham, P.S., Kawachi, H., Haydar, S.A., Chugh, S., Addona, T.A., Charron, K.B., Holzman, L.B., Shia, M., Shimizu, F., Salant, D.J. J. Clin. Invest. (1999) [Pubmed]
  39. Neph1 and nephrin interaction in the slit diaphragm is an important determinant of glomerular permeability. Liu, G., Kaw, B., Kurfis, J., Rahmanuddin, S., Kanwar, Y.S., Chugh, S.S. J. Clin. Invest. (2003) [Pubmed]
  40. Cloning and chromosomal localization of a human kidney cDNA involved in cystine, dibasic, and neutral amino acid transport. Lee, W.S., Wells, R.G., Sabbag, R.V., Mohandas, T.K., Hediger, M.A. J. Clin. Invest. (1993) [Pubmed]
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