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Chemical Compound Review:

Phosphocysteamine 2-aminoethylsulfanylphosphonic acid

Synonyms: CHEBI:74951, NSC-118587, AC1L1ERH, LS-65763, CTK8J4149, ...

Dragojević-Simić, V. et al., van't Hoff, W.G. et al., Neumann, H. et al., Vos, O. et al., Njoku, O.O. et al., et al.

Beckman, Mullin, Assadi,

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Disease relevance of Cystafos

Alkaline phosphatase [orthophosphoricmonoester phosphohydrolase (alkaline pH optimum), EC 3.1.3.1] purified from a Burkitt lymphoma cell line (Daudi) and Moloney-virus-induced murine leukemia (YAC) showed unique catalytic properties in substrate specificity and inhibition by cysteamine-S-phosphate [1].
Renal stones in nephropathic cystinosis treated with phosphocysteamine [2].
It has been reported that murine lymphoma APase does not cleave the S-P bond of CASP [3].

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

Fifty-nine patients with cystinosis were treated with cysteamine or phosphocysteamine in the United Kingdom up to May 1990 [7].

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

Compounds with a covered SH group, like WR 638, cysteamine phosphate, WR 2721, and AE 48527, showed practically no protection when dissolved in tissue-culture medium, but developed a protective activity when dissolved in rat blood [8].
The photolysis of cysteamine-S-phosphate gave cysteamine, inorganic orthophospate and taurine [9].

References

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]
Renal stones in nephropathic cystinosis treated with phosphocysteamine. Fischbach, M., Terzic, J., Cavalier, A., Mambrini, P., Geisert, J. Pediatr. Nephrol. (1997) [Pubmed]
A reinterpretation of phosphatase-N. Njoku, O.O., Early, J.A., Lumb, J.R. Oncodev. Biol. Med. (1982) [Pubmed]
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]
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]
Developmental toxicity of cysteamine in the rat: effects on embryo-fetal development. Beckman, D.A., Mullin, J.J., Assadi, F.K. Teratology (1998) [Pubmed]
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]
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]
Photoreactions of phosphorothioate and cysteamine-S-phosphate. Photosubstitution and photophosphoryl transfer. Neumann, H., Sokolovsky, M. Biochim. Biophys. Acta (1975) [Pubmed]

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