Statements in which the resource exists as a subject.
PredicateObject
rdf:type
lifeskim:mentions
pubmed:issue
4
pubmed:dateCreated
1984-6-7
pubmed:abstractText
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.
pubmed:language
eng
pubmed:journal
pubmed:citationSubset
IM
pubmed:chemical
pubmed:status
MEDLINE
pubmed:month
Apr
pubmed:issn
0007-0963
pubmed:author
pubmed:issnType
Print
pubmed:volume
110
pubmed:owner
NLM
pubmed:authorsComplete
Y
pubmed:pagination
439-49
pubmed:dateRevised
2004-11-17
pubmed:meshHeading
pubmed:year
1984
pubmed:articleTitle
Trichothiodystrophy: an electron-histochemical study of the hair shaft.
pubmed:publicationType
Journal Article