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Metachromatic leukodystrophy
Published in William L. Nyhan, Georg F. Hoffmann, Aida I. Al-Aqeel, Bruce A. Barshop, Atlas of Inherited Metabolic Diseases, 2020
The sulfatase enzyme is heat labile. A heat stable factor that increases activity several-fold is known as saposin B and, in rare instances, MLD results from defective activity of this protein, and arylsulfatase activity is normal [11, 12]. Deficiency of the heat stable factor causes deficiency of the enzymatic hydrolysis of sulfatide [13, 14]. Other variants with clinical MLD have signs of mucopolysaccharidosis and have been found to have multiple sulfatase deficiency (Chapter 99).
Retinoids in Keratinization Disorders
Published in Ayse Serap Karadag, Berna Aksoy, Lawrence Charles Parish, Retinoids in Dermatology, 2019
This uncommon X-linked recessive disorder occurs only in boys, although girl carriers can have or show mild desquamation. The lifelong condition affects about 1/2000–6000 boys with steroid sulfatase enzyme deficiency. Accumulation of cholesterol sulfate can result in retention hyperkeratosis. Lesions appear in the first year of life. There are larger and darker scales, particularly in flexural regions and to a lesser extent the extensor areas; however, palmoplantar regions are unaffected. Asymptomatic ocular opacities may appear in half of men and some female carriers. In laboratory analysis, cholesterol sulfate levels are increased, with elevated mobility of β-lipoproteins on electrophoresis. Steroid sulfatase enzyme levels are diminished or absent (14,15).
The Role of Light and Electromagnetic Fields in Maintaining Vascular Health
Published in Aruna Bakhru, Nutrition and Integrative Medicine, 2018
All of these bioactive molecules are inactive when sulfated, and this point has often been invoked to dismiss the utility of the sulfated versions to the body. However, I believe that one of their most important roles is to deliver sulfate to the tissues. When they arrive at their destination, a sulfatase detaches their sulfate and a sulfotransferase reattaches it to one of the sulfated GAGs in the extracellular matrix of the target cell, beefing up their sulfate supplies.
Metabolism of cyclic phenones in rainbow trout in vitro assays
Published in Xenobiotica, 2020
Jose Serrano, Mark A. Tapper, Richard C. Kolanczyk, Barbara R. Sheedy, Tylor Lahren, Dean E. Hammermeister, Jeffrey S. Denny, Michael W. Hornung, Alena Kubátová, Patricia A. Kosian, Jessica Voelker, Patricia K. Schmieder
To monitor for the potential formation of sulfate and glucuronide conjugates of hydroxylated metabolites, additional 450 µL exposure media samples (Cont, MC, MCS, ContS, and S) at each exposure time were hydrolyzed in vials at each exposure time with Sulfatase (40 units in 100 mM ammonium acetate buffer, pH 5.0 plus 5 mM D-saccharic acid-1,4-lactone) or β-Glucuronidase (48 units in 100 mM potassium phosphate buffer pH 6.8) for 12 h at 37 °C. Reactions were stopped with the addition of an equal volume of hexane. Concentrations of CPK, DPK, CBP and detected metabolites were measured by GC-MS as previously described for non-hydrolyzed exposure media and slices but using an additional DF of 1.26 (total DF = 1.29) corresponding to the addition of hydrolysis reagents. Experimental confirmation of Sulfatase and β-Glucuronidase enzyme activity is described in the Appendix.
Steroid sulfatase inhibitors: the current landscape
Published in Expert Opinion on Therapeutic Patents, 2021
Hanan S. Anbar, Zahraa Isa, Jana J. Elounais, Mariam A. Jameel, Joudi H. Zib, Aya M. Samer, Aya F. Jawad, Mohammed I. El-Gamal
In the human body, estrogen and androgen activity is regulated by sulfation and desulfation processes which are opposite to each other. Both processes are under the control of enzymatic activity including two main enzyme families represented as sulfotransferases and sulfatases. Their enzymatic activities result in changes in the structure and function of steroids, thyroids, xenobiotics, and neurotransmitters. Estrogens and androgens are mainly regulated by positive and negative feedback of these two enzymes. Steroid sulfatase (STS) converts the inactive metabolites estrone sulfate (E1S), estradiol sulfate, or 5-androstenediol sulfate into the active non-sulfated estrone (E1), estradiol, or 5-androstenediol, respectively. Aromatase and 17β-hydroxysteroid dehydrogenase type 7 (17β-HSD) are also involved in biosynthesis of estrone, estradiol, and 5-androstenediol (Figure 1). These enzymes are potential drug targets for the treatment of hormone-dependent cancers and other disorders. On the other hand, sulfotransferase converts the active form to inactive sulfated form[1]. Besides the known information and functions about STS, researchers have been more concerned about the metabolic effects and functions of STS and their contribution in the pathological pathways in some diseases including STS over-expression-related diseases; preeclampsia [2], hormone-dependent cancers such as breast cancer [3,4] prostate cancer [5], ovarian cancer [6], and other diseases or STS deficiency-related diseases such as X-linked ichthyosis [7], attention deficit hyperactivity disorder (ADHD) [8–10] and postpartum mood disorder [11,12].
The therapeutic effects of blocking IGF-R1 on mice model of skin cancer
Published in Journal of Dermatological Treatment, 2021
Glypican-3 is a type of cell surface heparan sulfate proteoglycan (HSPG). It is released from the HSPG by the action of sulfatase-2 enzyme. Glypican-3 is a member of the glypican family that enhances tumor cell invasion and metastasis (19). We have previously reported the increase of expression glypican-3 in experimentally induced skin cancer in mice and the antitumor activity of blocking glypican-3 activity (13). It is also proved to be overexpressed in many cancers such as hepatocellular carcinoma, lung cancer and others (20–22). Although PQ401 produced antitumor activity against skin cancer in our study, it did not affect the expression of glypican-3 as it is upstream of IGF-R1 in the cell signaling.