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Biochem Mol Biol Int ; 47(1): 137-42, 1999 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-10092953

RESUMO

The enzyme steroid sulfatase (STS) hydrolyses 3-beta-hydroxysteroid sulfates. The female-male STS activity ratio is 1.04-1.7:1 in several cell lines in adults and reaches 2:1 in prepubertal subjects. In fibroblasts, STS values in X-chromosome abnormalities show a partial positive correlation according to the number of X-chromosomes. X-linked ichthyosis (XLI) carriers, with only one copy of the STS gene, present lower STS levels than normal controls. This study analyzes the STS activity in leukocytes of 46,Xi(Xq); 45,X; XLI carriers and normal controls using 7-[3H]-dehydroepiandrosterone sulfate as substrate. X-monosomy (1.07 +/- 0.18 pmol/mg protein/h), Xq isochromosome (1.02 +/- 0.12 pmol/mg protein/h) and normal females (1.03 +/- 0.11 pmol/mg protein/h) had similar STS values (p > 0.05). XLI-carriers and males showed the lowest STS levels (0.34 +/- 0.04 pmol/mg protein/h, p < 0.001 and 0.82 +/- 0.14 pmol/mg protein/h, p < 0.05, respectively). Female-male STS activity ratio in leukocytes was 1.3:1. These data indicate that a complex mechanism regulates the STS expression depending on each type of cell line.


Assuntos
Arilsulfatases/sangue , Arilsulfatases/deficiência , Ictiose Ligada ao Cromossomo X/genética , Leucócitos/enzimologia , Aberrações dos Cromossomos Sexuais/enzimologia , Bandeamento Cromossômico , Feminino , Heterozigoto , Humanos , Isocromossomos , Masculino , Monossomia , Esteril-Sulfatase , Cromossomo X
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