Biomaterial Database

Plasma cyst(e)ine in homocyst(e)inemia. 1981

M H Malloy, and D K Rassin, and G E Gaull

Using a method developed to measure plasma protein-bound 1/2-cystine, we have observed that the metabolic state can affect the distribution of plasma cyst(e)ine between the bound and the free form. In five patients with homocyst(e)inemia, 1/2-homocystine was found to have replaced virtually all the cysteine normally present on sulfhydryl-binding sites of plasma proteins. The majority of the cyst(e)ine measured in the plasma of these patients (6.9 +/- 2.4 mumol/dl, mean +/- SD) was present in the form of a mixed disulfide with 1/2-homocystine (5.4 +/- 0.9 mumol/dl) rather than being free in the plasma or bound to plasma proteins.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D008715	Methionine	A sulfur-containing essential L-amino acid that is important in many body functions.	L-Methionine,Liquimeth,Methionine, L-Isomer,Pedameth,L-Isomer Methionine,Methionine, L Isomer
D011485	Protein Binding	The process in which substances, either endogenous or exogenous, bind to proteins, peptides, enzymes, protein precursors, or allied compounds. Specific protein-binding measures are often used as assays in diagnostic assessments.	Plasma Protein Binding Capacity,Binding, Protein
D001798	Blood Proteins	Proteins that are present in blood serum, including SERUM ALBUMIN; BLOOD COAGULATION FACTORS; and many other types of proteins.	Blood Protein,Plasma Protein,Plasma Proteins,Serum Protein,Serum Proteins,Protein, Blood,Protein, Plasma,Protein, Serum,Proteins, Blood,Proteins, Plasma,Proteins, Serum
D003545	Cysteine	A thiol-containing non-essential amino acid that is oxidized to form CYSTINE.	Cysteine Hydrochloride,Half-Cystine,L-Cysteine,Zinc Cysteinate,Half Cystine,L Cysteine
D003553	Cystine	A covalently linked dimeric nonessential amino acid formed by the oxidation of CYSTEINE. Two molecules of cysteine are joined together by a disulfide bridge to form cystine.	Copper Cystinate,L-Cystine,L Cystine
D005260	Female		Females
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