By John A. Sturman, Derek A. Applegarth (auth.), Alan A. Boulton, Glen B. Baker, James D. Wood (eds.)

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H , Rassm D. , and Gaul1 G. E (1981a) A method for the measurement of free and bound plasma cyst(e Anal Bzochem 113, 407415 Malloy M. , and Gaul1 G. E (1981b) Plasma cyst(e m homocyst(e)memra. Am J Clan Nutr 34, 2619-2621. , Laberge C , and Poter M (1983) Deficiency of Fumarylacetoacetase u-r the Acute Form of Hereditary Tyrosmemla Wrth Reference to Prenatal Diagnosis, in Pedlafrx Lzver Dzsease (Fisher M M. ), Plenum, New York. , and Fumeros S. (1975) A new approach for obtammg total tryptophan recovery in plasma samples deproteinrzed with sulphosalrcyllc acid 1 Chromafogr.

Hypermethionemla is frequently caused by mgestron of D,L-Met added to soy-based formulae that are deficient in Met The D form of the ammo acid is then seen as mcreased plasma and urinary Met values. Hypermethionemra also occurs in homocystinuria, and m this case both homocystme and its mixed disulfide with cystme should be seen m the amino acid patterns of plasma and urine. , 1983). Tyrosinemia is discussed below. 15. , 1981), and there are less well-characterized defects involving ormthmemia (see Stanbury et al , 1983).

B , Bogue D. C. and Anderson R. A (1960) Ion exchange chromatography of ammo acids Analysis of diffusion (mass transfer) mechamsms Anal Chem. 32, 1782-1792. Harrap K. , Smith C A , and Hill B. T. (1973) The occurrence of protein-bound mixed disulfides m rat tissues Bmch~m Bzophys. , and Delamater I’. V (1974) Reye’s syndrome and hyperammoacidemia. I Pedzaf 84, 362-365. Hirs C H. W , Moore S , and Stem W. , Moore S, and Stem W. H. , eds (1976) Tuurtne Raven, New York. , eds. (1982) Tutlrrne zn Nutrrtlon lmd Neurology Plenum, New York Jhaverl B M , Burst N.

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