Biomaterial Database

Determination of homocysteine, penicillamine, and their symmetrical and mixed disulfides by liquid chromatography with electrochemical detection. 1989

D L Rabenstein, and G T Yamashita

Department of Chemistry, University of California, Riverside 92521.

A method for the determination of D-penicillamine, homocysteine, homocystine, penicillamine-homocysteine mixed disulfide, and penicillamine disulfide in human plasma and urine is described. The method involves separation of the various thiols and disulfides by high-performance liquid chromatography with detection by a dual Hg/Au amalgam electrochemical detector. D-Penicillamine and homocysteine are detected at the downstream electrode; the disulfides are first reduced to thiols at the upstream electrode and then the thiols are detected at the downstream electrode. Hydrodynamic voltammograms were measured for the various thiols and disulfides to determine optimum settings for the electrochemical detector, and the effect of mobile phase parameters on retention times was studied to optimize the separation. A convenient method for the preparation of calibration solutions of penicillamine-homocysteine mixed disulfide by thiol/disulfide exchange with standardization of the solution by H NMR spectroscopy is described. Detection limits are below the concentrations of homocystine and penicillamine-homocysteine mixed disulfide reported to be present in the plasma and urine, respectively, of homocystinuric patients under treatment with D-penicillamine.

UI	MeSH Term	Description	Entries
D010084	Oxidation-Reduction	A chemical reaction in which an electron is transferred from one molecule to another. The electron-donating molecule is the reducing agent or reductant; the electron-accepting molecule is the oxidizing agent or oxidant. Reducing and oxidizing agents function as conjugate reductant-oxidant pairs or redox pairs (Lehninger, Principles of Biochemistry, 1982, p471).	Redox,Oxidation Reduction
D010396	Penicillamine	3-Mercapto-D-valine. The most characteristic degradation product of the penicillin antibiotics. It is used as an antirheumatic and as a chelating agent in Wilson's disease.	Dimethylcysteine,Mercaptovaline,beta,beta-Dimethylcysteine,Copper Penicillaminate,Cuprenil,Cuprimine,D-3-Mercaptovaline,D-Penicillamine,Metalcaptase,D 3 Mercaptovaline,D Penicillamine,Penicillaminate, Copper,beta,beta Dimethylcysteine
D002851	Chromatography, High Pressure Liquid	Liquid chromatographic techniques which feature high inlet pressures, high sensitivity, and high speed.	Chromatography, High Performance Liquid,Chromatography, High Speed Liquid,Chromatography, Liquid, High Pressure,HPLC,High Performance Liquid Chromatography,High-Performance Liquid Chromatography,UPLC,Ultra Performance Liquid Chromatography,Chromatography, High-Performance Liquid,High-Performance Liquid Chromatographies,Liquid Chromatography, High-Performance
D004220	Disulfides	Chemical groups containing the covalent disulfide bonds -S-S-. The sulfur atoms can be bound to inorganic or organic moieties.	Disulfide
D004563	Electrochemistry	The study of chemical changes resulting from electrical action and electrical activity resulting from chemical changes.	Electrochemistries
D004566	Electrodes	Electric conductors through which electric currents enter or leave a medium, whether it be an electrolytic solution, solid, molten mass, gas, or vacuum.	Anode,Anode Materials,Cathode,Cathode Materials,Anode Material,Anodes,Cathode Material,Cathodes,Electrode,Material, Anode,Material, Cathode
D006710	Homocysteine	A thiol-containing amino acid formed by a demethylation of METHIONINE.	2-amino-4-mercaptobutyric acid,Homocysteine, L-Isomer,2 amino 4 mercaptobutyric acid,Homocysteine, L Isomer,L-Isomer Homocysteine
D006711	Homocystine
D006712	Homocystinuria	Autosomal recessive inborn error of methionine metabolism usually caused by a deficiency of CYSTATHIONINE BETA-SYNTHASE and associated with elevations of homocysteine in plasma and urine. Clinical features include a tall slender habitus, SCOLIOSIS, arachnodactyly, MUSCLE WEAKNESS, genu varus, thin blond hair, malar flush, lens dislocations, an increased incidence of MENTAL RETARDATION, and a tendency to develop fibrosis of arteries, frequently complicated by CEREBROVASCULAR ACCIDENTS and MYOCARDIAL INFARCTION. (From Adams et al., Principles of Neurology, 6th ed, p979)	CBS Deficiency,Cystathionine beta-Synthase Deficiency Disease,Cystathionine Beta Synthase Deficiency,Deficiency Disease, Cystathionine beta-Synthase,CBS Deficiencies,Cystathionine beta Synthase Deficiency Disease,Deficiencies, CBS,Deficiency Disease, Cystathionine beta Synthase,Deficiency, CBS
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man