| Autor: |
Gummer, C.L., Dawber, R.P.R., Price, V.H. |
| Předmět: |
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| Zdroj: |
British Journal of Dermatology; Apr1984, Vol. 110 Issue 4, p439-449, 11p |
| Abstrakt: |
We have used sensitive electron-histochemical methods to study the subtle ultrastructural variations of cystine incorporation into the hair shaft in trichothiodystrophy. We have shown a general reduction in the cystine (sulphur) content of both the cuticle and the cortex. Discontinuity, and in some cases, complete absence of the cuticular A-layer results in premature weathering of the cuticle and weakening of the hair shaft. The ultrastructural findings support the work of Gillespie & Marshall (1981) in demonstrating the absence or re-characterization of the high sulphur matrix proteins and show further evidence for the incorporation, and abnormal distribution and deposition of sulphur-rich proteins in the hair cortex and cuticle. We conclude that the similar yet different results obtained from each patient's hair sample are characteristic of trichothiodystrophy, a neuro-ectodermal symptom complex which may represent a final common pathway of more than one metabolic disturbance. [ABSTRACT FROM AUTHOR] |
| Databáze: |
Complementary Index |
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