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Chemical Compound Review

Phosphocysteamine     2-aminoethylsulfanylphosphonic acid

Synonyms: CHEBI:74951, NSC-118587, AC1L1ERH, LS-65763, CTK8J4149, ...
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Disease relevance of Cystafos


High impact information on Cystafos

  • Most importantly, in lymphoblasts derived from INCL patients, phosphocysteamine, a known lysosomotrophic drug, mediates the depletion of lysosomal ceroids, prevents their re-accumulation and inhibits apoptosis [4].
  • Influence of a radioprotector WR-638 on the lymphoid compartment of the irradiated rat thymus: a flow cytometric analysis [5].
  • The T cell composition of the thymus of X-ray irradiated (3.5 Gy) Wistar rat protected with WR-638 was analyzed by flow cytometry using monoclonal antibodies directed to the Thy 1.1, CD43, CD2, CD5, CD4, CD8 and class I and II MHC antigens [5].
  • Pregnant rats were given cysteamine (as phosphocysteamine) from day 6.5 through day 18.5 postconception and fetuses were assessed for survival, growth, and structural abnormalities on day 20 [6].
  • The mean final doses of cysteamine (33 mg/kg per day) and phosphocysteamine (37 mg/kg per day base equivalent) were less than the mean dose (51 mg/kg per day) used in a United States multicentre trial [7].

Chemical compound and disease context of Cystafos


Biological context of Cystafos

  • Neumann shows similar data to ours in terms of the 3-fold ratio, but considers all hydrolysis of CASP as representative of 'normal' APase [3].

Associations of Cystafos with other chemical compounds


  1. Comparative study of alkaline phosphatase activity in lymphocytes, mitogen-induced blasts, lymphoblastoid cell lines, acute myeloid leukemia, and chronic lymphatic leukemia cells. Neumann, H., Klein, E., Hauck-Granoth, R., Yachnin, S., Ben-Bassat, H. Proc. Natl. Acad. Sci. U.S.A. (1976) [Pubmed]
  2. Renal stones in nephropathic cystinosis treated with phosphocysteamine. Fischbach, M., Terzic, J., Cavalier, A., Mambrini, P., Geisert, J. Pediatr. Nephrol. (1997) [Pubmed]
  3. A reinterpretation of phosphatase-N. Njoku, O.O., Early, J.A., Lumb, J.R. Oncodev. Biol. Med. (1982) [Pubmed]
  4. Lysosomal ceroid depletion by drugs: therapeutic implications for a hereditary neurodegenerative disease of childhood. Zhang, Z., Butler, J.D., Levin, S.W., Wisniewski, K.E., Brooks, S.S., Mukherjee, A.B. Nat. Med. (2001) [Pubmed]
  5. Influence of a radioprotector WR-638 on the lymphoid compartment of the irradiated rat thymus: a flow cytometric analysis. Dragojević-Simić, V., Colić, M., Gasić, S. Int. J. Radiat. Biol. (1994) [Pubmed]
  6. Developmental toxicity of cysteamine in the rat: effects on embryo-fetal development. Beckman, D.A., Mullin, J.J., Assadi, F.K. Teratology (1998) [Pubmed]
  7. The treatment of cystinosis with cysteamine and phosphocysteamine in the United Kingdom and Eire. van't Hoff, W.G., Gretz, N. Pediatr. Nephrol. (1995) [Pubmed]
  8. In vitro evaluation of some latent radioprotective compounds. Vos, O., Budke, L., Grant, G.A. Int. J. Radiat. Biol. Relat. Stud. Phys. Chem. Med. (1976) [Pubmed]
  9. Photoreactions of phosphorothioate and cysteamine-S-phosphate. Photosubstitution and photophosphoryl transfer. Neumann, H., Sokolovsky, M. Biochim. Biophys. Acta (1975) [Pubmed]
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