PHYSICOCHEMICAL PROPERTIES OF HAIR IN BIDS SYNDROME

  • 1 January 1976
    • journal article
    • research article
    • Vol. 28  (5) , 514-521
Abstract
The physicochemical properties of hair from a new [human] recessive syndrome associated with brittle hair, intellectual impairment, decreased fertility, and short stature were studied. Electrophoresis of the SCM[S-carboxymethyl]-structural proteins showed that the .alpha.-polypeptides appeared normal, but the matrix component was markedly reduced. This was confirmed by finding a normal .alpha.-X-ray diffraction pattern but a reduced 1/2 cystine content of hair and an abnormal stress-strain curve. EM studies revealed extreme disorganization of the filaments which most likely resulted from the absence of normal cross-linking. Nails, which contain structural proteins similar to hair, also showed the abnormality. Since the matrix component seen by electrophoresis consists of more than 1 component the defect cannot be explained as a single structural gene abnormality.