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Fabry's disease: biochemical and histochemical studies on hair roots for carrier detection
Author(s) -
VERMORKEN A. J. M.,
WETERINGS P. J. J. M.,
SPIERENBURG G. Th.,
BENNEKOM C. A.,
WIRTZ P.,
BRUYN C. H. M. M.,
OEI T. L.
Publication year - 1978
Publication title -
british journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.304
H-Index - 179
eISSN - 1365-2133
pISSN - 0007-0963
DOI - 10.1111/j.1365-2133.1978.tb01621.x
Subject(s) - acid phosphatase , alkaline phosphatase , phosphatase , enzyme , biochemistry , fabry disease , duct (anatomy) , enzyme assay , biology , chemistry , anatomy , pathology , medicine , disease
SUMMARY A method to assay α‐galactosidase and acid phosphatase activities in single hair roots is described. Enzyme histochemical studies show that the distribution of acid phosphatase in the human hair root matches that of α‐galactosidase. Histochemically, the main activity is located in the upper part of the sheath near the orifice of the duct of the sebaceous gland. This is confirmed by enzyme assays on different parts of the hair root after dissection. The variation in the values found in individual hair roots is improved by relating α‐galactosidase to acid phosphatase activities. Storage experiments indicate a remarkable stability of both α‐galactosidase and acid phosphatase in human hair roots.