Proteins of normal hair and of cystine-deficient hair from mentally retarded siblings.
1. S-Carboxymethylkerateines extracted from normal hair can be fractionated into high-sulphur and low-sulphur proteins similar to those obtained from sheep's wool. Normal human hair gives a major high-sulphur protein of higher molecular weight and S-carboxymethylcysteine content than any isolated from normal sheep's wool. 2. The proteins from cystine-deficient hair can also be divided into high-sulphur and low-sulphur proteins. There is a lower proportion of high-sulphur protein in cystine-deficient hair than in normal hair. 3. The high-sulphur proteins from cystine-deficient hair have an abnormal amino acid composition and in particular are lower in S-carboxymethylcysteine content than the corresponding proteins from normal hair. New components are present and the content of very high-sulphur proteins of high molecular weight is much decreased. The low-sulphur proteins of cystine-deficient hair are probably also deficient in S-carboxymethylcysteine. 4. The proteins of cystine-deficient hair probably resemble those in the normal hair root, except that disulphide-bridge formation has occurred.
D000179:Acrylates; D000596:Amino Acids / Q000032:analysis; D000818:Animals; D002848:Chromatography, DEAE-Cellulose; D003553:Cystine / Q000032:analysis*; D004586:Electrophoresis; D005260:Female; D005782:Gels; D006197:Hair / Q000032:analysis*; D006801:Humans; D006868:Hydrolysis; D008607:Intellectual Disability / Q000235:genetics / Q000378:metabolism*; D007525:Isoelectric Focusing; D008970:Molecular Weight; D009905:Optical Rotatory Dispersion; D011506:Proteins / Q000032:analysis*; D012756:Sheep; D013455:Sulfur / Q000032:analysis*; D014316:Tritium; D014935:Wool / Q000032:analysis
D000179:Acrylates; D000596:Amino Acids; D005782:Gels; D011506:Proteins; D014316:Tritium; D003553:Cystine; D013455:Sulfur