The physicochemical properties of hair in the BIDS syndrome.

The physicochemical properties of hair from a new recessive syndrome associated with brittle hair, intellectual impairment, decreased fertility, and short stature have been studied. Electrophoresis of the SCM-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. Electron-microscopic 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 one component the defect cannot be explained as a single structural gene abnormality.

Citation

H P Baden, C E Jackson, L Weiss, K Jimbow, L Lee, J Kubilus, R J Gold. The physicochemical properties of hair in the BIDS syndrome. American journal of human genetics. 1976 Sep;28(5):514-21

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PMID: 984047

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