Inborn Errors of Metabolism the Croonian Lectures Delivered Before the Royal Co
Inborn Errors of Metabolism the Croonian Lectures Delivered Before the Royal Co
Archibald Edward Garrod
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NH 2, CH. NH 2 CH 2 . NH 2 CO. OH CO. OH. CO. OH. Cystin. Cysteic acid. Taurin. and it has actually been obtained from it, in vitro, by Friedmann. 28 It is obvious that the cystin destined to follow this metabolic path must be set apart at a very early stage of catabolism, before either the sulphur atom or the amino-group has been removed, and there is evidence that the removal of both of these is early effected. The question whether the taurin, as such, com- bines directly with cholalic acid t...o form taurocholic acid, or whether the combination first occurs with cystin itself or with some other precursor of taurin, taurocholic acid being afterwards produced by oxidation, is still an open one. In favour of the latter view may be quoted an observation made by L. Blum 29 that when cystin is injected into the portal circulation of an animal with a biliary fistula the sulphur of the bile is thereby increased, but that the additional sulphur is neither in the form of cystin nor in that of taurin.
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