X-linked Ichthyosis - Physiology and Biochemistry

Physiology and Biochemistry

The STS enzyme (EC 3.1.6.2), also referred to as Arylsulfatase C, is expressed throughout the body, with highest expression in the skin, liver, lymph nodes, and placenta, and lower expression in breast tissue and brain STS catalyzes the hydrolysis of sulfated steroids, such as estrone sulfate and dehydroepiandrosterone sulfate (DHEAS), to non-sulfated steroids estradiol and androstenediol, respectively. Prenatally, the enzyme is involved in plancental estrogen production. The enzyme is also involved in adrenal steroid production as well as conversion of sulfated steroids in other tissues.

There seems to be a particularly important role for the enzyme in skin. Deficiency of the enzyme leads to the characteristic dry and scaly skin seen in ichthyosis. Recent research indicates that the skin abnormalities seen in XLI may be due to accumulation of cholesterol sulfate in the outer epidermis, leading to abnormal barrier function and corneocyte retention.

Read more about this topic:  X-linked Ichthyosis

Famous quotes containing the words physiology and and/or physiology:

    Now the twitching stops. Now you are still. We are through with physiology and theology, physics begins.
    Alfred Döblin (1878–1957)

    The world moves, but we seem to move with it. When I studied physiology before ... there were two hundred and eight bones in the body. Now there are two hundred and thirty- eight.
    Ellen Henrietta Swallow Richards (1842–1911)