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PubMed1981

Formation of pyrophosphate, tripolyphosphate, and phosphorylimidazole with the thioester, n, s-diacetyl-cysteamine, as the condensing agent.

Reaction of 0.20M orthophosphate with 0.20M N,S-diacetylcysteamine in 0.40M imidazole at pH 7.0 or 8.0 under drying conditions at 50 degree C for 6 days yields pyrophosphate and tripolyphosphate in the presence and absence of 0.10M divalent metal ion. The efficiency of utilization of N,S-diacetylcysteamine in the formation of pyrophosphate linkages ranges from 3 - 8% under the above conditions. The thioester, N,S-diacetylcysteamine, and imidazole are required for phosphoanhydride formation. Reaction of 0.40M orthophosphate with 0.20M N,S-diacetylcysteamine in 0.40M orthophosphate with 0.20M N,S-diacetylcysteamine in 0.40M imidazole at ambient temperature for 6 days yields phosphorylimidazole in the absence or presence of 0.50M MgCl2. Phosphorylimidazole and pyrophosphate are formed in the presence of 0.05M CaCl2; pyrophosphate and tripolyphosphate are formed with 0.15M CaCl2. The efficiency of utilization of N,S-diacetylcysteamine in the formation of pyrophosphate linkages is roughly 7% at 6 days of reaction with 0.15M CaCl2. The thioester, N,S-diacetylcysteamine and imidazole are required for the formation of phosphoanhydrides. The significance of these reactions to molecular evolution is discussed.

Biological Evolution↗
PubMed1983

Nephropathic cystinosis: ineffectiveness of cysteamine therapy for ocular changes.

The biochemical hallmark of nephropathic cystinosis is the intralysosomal accumulation of free cystine in various organs, including the conjunctiva, cornea, bone marrow, leukocytes, lymph nodes, and internal organs. A patient with the infantile form of nephropathic cystinosis develops several renal tubular malfunctions during the first year of life; these eventually lead to end-stage renal failure and eventual death by the time the patient is 10 years of age. Ocular changes are prominent and may be so typical that an early diagnosis can be achieved by an ophthalmologic examination before the nephropathic signs become evident.

Child↗