Steroid Sulfatase Deficiency

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چکیده

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منابع مشابه

Development of steroid sulfatase inhibitors.

Hydrolysis of biologically inactive steroid sulfates to unconjugated steroids by steroid sulfatase (STS) is strongly implicated in rendering estrogenic stimulation to hormone-dependent cancers such as those of the breast. Considerable progress has been made in the past two decades with regard to the discovery, design and development of STS inhibitors. We outline historical aspects of their deve...

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Testicular steroid sulfatase. Substrate specificity and inhibition.

Kinetic studies of the cleavage of dehydroepiandrosterone-sulfate (i) and androstenediol-3-sulfate by a particulate enzyme preparation from a rat testicular microsomal fraction gave K m values of 2.04 x 10-6M for DHA-S and 0.935 x 10-6M for androstenediol-3-sulfate with identical Vma x values° Inhibition studies with equimolar concentrations of substrate and inhibitor demonstrated that 5~-andro...

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Steroid sulfatase gene in XX males.

The human X and Y chromosomes pair and recombine at their distal short arms during male meiosis. Recent studies indicate that the majority of XX males arise as a result of an aberrant exchange between X and Y chromosomes such that the testis-determining factor gene (TDF) is transferred from a Y chromatid to an X chromatid. It has been shown that X-specific loci such as that coding for the red c...

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Steroid sulfatase: molecular biology, regulation, and inhibition.

Steroid sulfatase (STS) is responsible for the hydrolysis of aryl and alkyl steroid sulfates and therefore has a pivotal role in regulating the formation of biologically active steroids. The enzyme is widely distributed throughout the body, and its action is implicated in physiological processes and pathological conditions. The crystal structure of the enzyme has been resolved, but relatively l...

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High levels of oxysterol sulfates in serum of patients with steroid sulfatase deficiency.

Steroid sulfatase (STS) deficiency is the underlying cause of the skin condition known as recessive X-linked ichthyosis (RXLI). RXLI patients show scales on their skin caused by high concentrations of cholesterol sulfate (CS), as they are not capable of releasing the sulfate group from its structure to obtain free cholesterol. CS has been reported, so far, as the sole sulfated steroid with incr...

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ژورنال

عنوان ژورنال: Pediatric Research

سال: 1977

ISSN: 0031-3998,1530-0447

DOI: 10.1203/00006450-197708000-00008