| 释义 |
porphobilinogen Biochem.|ˌpɔːfəʊbaɪˈlɪnəgən|
[a. G. porphobilinogen (Waldenström & Vahlquist 1939, in Zeitschr. f. physiol. Chem. CCLX. 191), f. prec.: see -ogen.]
A colourless, crystalline, substituted pyrrole, C10H14N2O4 (see quot. 1972), which in animals is a precursor of porphyrins and is excreted in the commoner forms of porphyria.
1939Chem. Abstr. XXXIII. 8777/1 In acute porphyria the urine contains a colorless substance (porphobilinogen)..which is a pyrrole deriv. and is converted to uroporphyrin on standing. 1961Lancet 22 July 175/2 The clinical signs disappeared quickly, but the patient continued to excrete rather a lot of porphobilinogen and uroporphyrin in the urine. 1964A. White et al. Princ. Biochem. (ed. 3) xlii. 790 Enzymic preparations utilizing porphobilinogen for porphyrin synthesis have been isolated from plant, bacterial, and animal sources. 1970R. W. McGilvery Biochem. xxi. 496 The parent indole ring is made in the next step by the condensation of two molecules of 5-aminolevulinate to form porphobilinogen. 1972Stedman's Med. Dict. (ed. 22) 1004/2 Porphobilinogen,..2-aminomethyl-4-(21-casboxyethyl)-3-carboxymethylpyrrole.
Hence ˌporphobiˌlinogeˈnuria, the presence of porphobilinogen in the urine.
1961Ann. Rev. Med. XII. 265 The probable relationship between this syndrome [sc. toxic porphyria] and the consumption of seed wheat treated with hexachlorobenzene-containing fungicides is supported by the production of massive uroporphyrinuria and porphobilinogenuria in rats by mixing hexachlorobenzene with the food. 1970Passmore & Robson Compan. Med. Stud. II. xxxi. 4/1 In hepatic porphyria of Swedish type, new methods of measuring aminolaevulinic acid and porphobilinogen enable subclinical cases to be detected. Such cases are said to have a low degree of expressivity by comparison with their relatives who have obvious clinical manifestations and readily detectable porphobilinogenuria. |