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PubMed1985

[Changes in placental enzymatic activities in relation to estrogen production during pregnancy].

To study the mechanism of production of estrogen during pregnancy, the following in vitro and in vivo studies were undertaken; 1) Weight of human placenta and total estradiol (E2) levels in the maternal peripheral vein were measured at different weeks of gestation. 2) Changes in E2 levels after DHA-S 100mg loading were calculated at the 1st, 2nd and 3rd trimesters. 3) Steroid enzyme activities including sulfatase, 3 beta-HSD and aromatase in placenta obtained in the 1st, 2nd and 3rd trimesters were measured. Results were as follows; 1) The weight of the human placenta increased gradually as gestation progressed. Twofold placental weight was noticed from the 2nd to the 3rd trimester. 2) Total E2 in the maternal peripheral vein increased steadily, being 4.07 +/- 1.74 ng/ml at the 1st trimester, 27.72 +/- 11.67 ng/ml and 104.12 +/- 57.89 ng/ml at the 2nd and 3rd trimesters respectively. The increase in E2 from the 2nd to the 3rd trimester was greater than that of the placental weight. 3) Increases in E2 following DHA-S loading were 1.01 ng/ml at the 1st, 29.2 ng/ml at the 2nd and 98.2 ng/ml at the 3rd trimester. 4) No significant differences were observed between placental 3 beta-HSD and sulfatase activities in the placenta obtained at the three different stages of pregnancy, while aromatase activity was found to be significantly higher in the placenta of the 3rd trimester than that of 2nd trimester. These results indicate that the remarkable increase in estrogen production in the 3rd trimester may be explained partially by increased aromatizing enzyme activity in the placenta.

Dehydroepiandrosterone↗
PubMed1995

[Metachromatic leukodystrophy (MLD) and Multiple sulphatase deficiency (MSD)].

MLD is caused by a deficiency of arylsulfatase A and hence sulfolipids are accumulated in various patient's tissues. Various clinical phenotypes including activator deficiency, pseudodeficiency and MSD. Recently, molecular basis of these disorders have been identified. Clinical phenotype in MLD is well correlated with their genotype. Most common mutation in Caucasian MLD is caused by 609A mutation which produces late infantile MLD. In Japanese, most common mutation is 445A mutation. Essential treatment of MLD is recently carried out by bone marrow transplantation. On the hand, MSD is caused by multiple deficiencies of various sulfatases and hence accumulated various sulfated compounds such as sulfatide and acid mucopolysaccharides. The clinical features have combined characteristics of MLD and mucopolysaccharidosis. The cause of this disorder is recently identified as abnormal modification of various sulfatase.

Base Sequence↗
PubMed1998

Two novel mutations of the arylsulfatase B gene in two Italian patients with severe form of mucopolysaccharidosis. Mutations in brief no. 127. Online.

Mucopolysaccharidosis type VI (MPS VI) or Maroteaux-Lamy syndrome, is a autosomal recessive disorder, due to the deficiency of the lysosomal enzyme N-acetylgalactosamine-4-sulfatase (arylsufatase B, ASB: EC 3.1.6.12). Three classical forms of the disease have been differentiated: severe, intermediate, mild. Mutational analysis of the ASB gene resulted in the identification of 30 ASB mutant alleles, each of which was found to be unique among unrelated patients, demonstrating a broad molecular heterogeneity of the disease. In this communication we present two novel mutant alleles in two severely affected subjects. Both alterations, the missense mutation G302R and the nonsense Q456X, were found in homozygosity and were confirmed by amplification refractory mutation system (ARMS) or restriction analysis. The missense G302R mutation concerns an amino acid which may be of special importance to the polypeptide, since 302 position is completely conserved in all the eukaryotic sulfatases aligned so far; the nonsense mutation Q456X leads to the translation of a putative mutant ASB protein lacking the last 78 amino acids with a loss of the 8 kD mature polypeptide, one of the two peptides generated by intralysosomal proteolytic processing of the 64kD precursor.

Alleles↗
PubMed1983

Deficiency of lysosomal hydrolases in apparently healthy individuals.

The deficiency of a lysosomal hydrolase usually results in the storage of its substrate(s) leading to various clinical abnormalities, typical for each deficiency. However, in certain lysosomal hydrolases, an apparent deficiency was noted which does not result in the classical clinical picture. This condition was described for aryl sulfatase A, beta-hexosaminidase, alpha-galactosidase, and galactocerebrosidase, where apparently healthy individuals showed in vitro very low hydrolase activity, usually indistinguishable from the affected patients. The deficiency was usually observed with both the synthetic and natural substrates. In the case of aryl sulfatase A deficiency, no clinical abnormalities were noted in these individuals, and cultured cells obtained from them were able to catabolize normally the natural substrate. Such cases are therefore referred as pseudodeficient. In other cases, such as in beta-hexosaminidase-A deficiency, mild manifestations of the corresponding disorder were reported with subsequent intralysosomal storage of GM2 ganglioside. Our analysis indicates that most of these cases represent a compound heterozygote for the deficient allele and another allele coding for an in vitro low enzyme activity (pseudodeficiency). A complete biochemical explanation for this phenomena is not yet established. The importance of understanding this condition(s) for proper genetic counseling is discussed.

Adult↗
PubMed1995

X-linked ichthyosis without STS deficiency: clinical, genetical, and molecular studies.

We report on a Sardinian pedigree with congenital ichthyosis associated with normal levels of steroid sulfatase and a normal molecular pattern, as detectable with a cDNA probe for the steroid sulfatase (STS) gene. Though the pattern of transmission of the disease is consistent with X-linked recessive inheritance, this form of ichthyosis was found to segregate independently of genetic polymorphisms detected by probes of the region Xp22.3, where the STS locus has been mapped. The search for close genetic linkages with other polymorphic markers scattered along the entire X chromosome has so far been fruitless. For the time being, the main conclusion derived from these data is that STS deficiency is not a sine qua non for X-linked ichthyosis which may also result from a mutational event at an X-chromosomal site genetically unlinked to the STS locus.

Arylsulfatases↗
PubMed2001

Involvement of up-regulation of 17beta-hydroxysteroid dehydrogenase type 1 in maintenance of intratumoral high estradiol levels in postmenopausal breast cancers.

Estradiol (E2) and estrone (E1) levels as well as mRNA expression levels of aromatase, sulfatase and 17beta-hydroxysteroid dehydrogenase type 1 (17beta-HSD1) in breast cancer tissues were studied to elucidate the mechanism involved in the maintenance of the intratumoral high E2 levels in postmenopausal patients with very low serum E2 levels. Intratumoral E2 levels of postmenopausal patients (127.2 +/- 17.5 pg/g) (mean +/- SE) were not significantly different from those of premenopausal patients (110.1 +/- 10.1 pg/g) (p = 0.36). The mRNA expression levels of aromatase and sulfatase, determined by a quantitative real-time PCR, were not significantly different between premenopausal and postmenopausal breast cancers, but 17beta-HSD1 mRNA expression levels were significantly higher in postmenopausal than premenopausal breast cancers (p < 0.05). Intratumoral E2/E1 ratios were significantly higher in postmenopausal than premenopausal breast cancers (p < 0.01). These results demonstrate that the increased conversion from E1 to E2 catalyzed by 17beta-HSD1 may play an important role in the maintenance of the intratumoral high E2 levels in postmenopausal patients.

17-Hydroxysteroid Dehydrogenases↗
PubMed1987

Ultrastructural localization of lysosomal enzymes in the egg cortex of Brachydanio.

The localization of acid phosphatase (E.C. 3.1.3.2), inorganic trimetaphosphatase (E.C. 3.6.1.2), and aryl sulfatase (E.C. 3.1.6.1) in the cortex of unactivated and activated eggs of Brachydanio was examined by ultrastructural cytochemistry. Using a lead capture method, activity for all three acid hydrolases was demonstrated in organelles of the cortex before and after egg activation. Acid phosphatase (AcPase) reaction product was consistently present in primary lysosomes, secondary lysosomes, multivesicular bodies, and yolk bodies. AcPase activity was absent from mitochondria, profiles of the endoplasmic reticulum, coated pits of exocytosed cortical granules, and coated vesicles. Although most cortical granules of the mature, unactivated egg were unreactive for this enzyme, a few showed AcPase reaction product. It is not clear whether the AcPase-positive granules might be an immature form of cortical granules or a subpopulation of these organelles with lysosomal properties. Most cisternae of the Golgi apparatus did not stain for AcPase; however, reaction product was occasionally localized in a single cisterna as well as several small vesicles at the inner face of the Golgi. The intensity of the reaction product and the pattern of distribution of trimetaphosphatase (Tm-Pase) activity was very similar to that of AcPase. However, TmPase was never observed in cortical granules. Cortices of unactivated and activated eggs showed less overall aryl sulfatase (ArSase) activity when compared with AcPase and TmPase. The presence of ArSase reaction product in lysosomes and multivesicular bodies confirmed the acid hydrolytic nature of these organelles. AcPase and TmPase, and to a lesser extent ArSase, are adequate markers of a cortical lysosomal system in the danio egg.(ABSTRACT TRUNCATED AT 250 WORDS)

Acid Anhydride Hydrolases↗
PubMed1983

Effect of castration and steroid treatments on the activity of some hydrolytic enzymes in dog prostate.

Adult mongrel dogs were castrated and treated by intramuscular injections of 5 alpha-androstane-3 alpha,17 beta-diol (androstanediol) alone or in combination with estradiol in order to find convenient enzymatic markers of hormone action in prostate. The activities of 15 hydrolytic enzymes were determined. Arginine esterase, acid sulfatase, and acid phosphatase were found to be the most sensitive markers of testicular hormones since they were decreased 18-, 5- and 5-fold respectively after 1 month of castration. The enzyme activities returned to precastration levels after 2 weeks of injection of androstanediol to castrated animals. The effect of androstanediol on the majority of the remaining enzymes was small. In general, the activities obtained after androstanediol treatment in combination with estradiol were similar to those obtained with androstanediol alone. Finally, beta-glucuronidase and neutral sulfatase were increased after castration, a finding that suggests that these enzymes are constituents of stromal cells. These studies will provide a basis for future studies of hormone action in the dog prostate.

Acid Phosphatase↗
PubMed1995

Dehydroepiandrosterone concentration in breast cancer tissue is related to its plasma gradient across the mammary gland.

Dehydroepiandrosterone (DHEA) has been shown to affect the growth of mammary carcinomas both in vitro and in vivo. In humans, very high levels of DHEA and/or dehydroepiandrosterone sulfate (DHEAS) have been found in breast tissues and secretions, and epidemiological studies suggest a role of these steroids in the modulation of breast cancer growth. An uptake from plasma and a transformation from precursors can be both postulated, but the main source of the adrenal C19 steroids found within the breast is debated. Attempting to clarify this point, in ten patients undergoing surgery for breast cancer we studied: a) DHEAS and DHEA concentrations in tumor tissue; b) the differences between DHEAS (or DHEA) concentration in peripheral venous plasma and that draining the affected breast, that we assume to reflect the arteriovenous gradient of these steroids; c) DHEA sulfatase activity in tumor tissue. Results show that DHEA sulfatase activity is not related to DHEAS or DHEA concentrations in breast cancer tissue. A negative DHEA plasma gradient across the breast is unveiled, whereas DHEAS levels are not different in blood supplying and draining the breast with cancer. The DHEA plasma gradient across the breast is positively related to DHEA concentration in tumor tissue. Data are consistent with the hypothesis that the plasma source contributes remarkably to DHEA found within breast cancer tissue.

Adult↗
PubMed1993

Lysosomal sulfate efflux following glycosaminoglycan degradation: measurements in enzyme-supplemented Maroteaux-Lamy syndrome fibroblasts and isolated lysosomes.

Studies using lysosomal membrane vesicles have suggested that efflux of the sulfate that results from lysosomal glycosaminoglycan degradation is carrier-mediated. In this study, glycosaminoglycan degradation and sulfate efflux were examined using cultured skin fibroblasts and lysosomes deficient in the lysosomal enzyme N-acetylgalactosamine-4-sulfatase. Such fibroblasts store dermatan sulfate lysosomally, which could be labelled biosynthetically with Na2(35)SO4. The addition of recombinant N-acetylgalactosamine-4-sulfatase to the media of 35S labelled fibroblasts degraded up to 82% of the stored dermatan [35S] sulfate over a subsequent 96 h chase and released inorganic [35S] sulfate into the medium. In the presence of 4-acetamido-4'-isothiocyanatostilbene-2,2'-disulfonic acid (SITS), sulfate was reused to a minor extent in newly synthesized proteoglycan. Isolated granules from recombinant enzyme supplemented fibroblasts degraded stored dermatan [35S]sulfate to sulfate which was rapidly released into the medium at a rate that was reduced by the extra-lysosomal presence of the lysosomal sulfate transport inhibitors SITS, Na2SO4 and Na2MoO4. SITS also inhibited dermatan sulfate turnover, although it had no effect on the action of purified recombinant enzyme in vitro. These data imply that sulfate clearance occurred concomitantly with dermatan sulfate turnover in the lysosome even at high substrate loading, and that lysosome-derived sulfate, while available, is reutilized minimally in synthetic pathways.

Cells, Cultured↗
PubMed1975

Action of the mycotoxin of Fusarium sporotrichiella v. sporotrichioides on lysosomal membranes.

The effect of the mycotoxin of Fusarium sporotrichiella v. sporotrichioides (sporofusarin) was studied in vitro on the total and nonsedimenting activity of eight lysosomal enzymes: acid ribonuclease, aryl sulfatases A and B, beta-glucuronidase, alpha- and beta-galactosidases, beta-glucosidase, beta-acetylglucosaminidase, and alpha-mannosidase. Incubation of a suspension of rat liver lysosomes with an aqueous solution of sporofusarin led to inhibition of the total activity of the membrane-bound lysosomal enzyme beta-glucosidase. In a dose of only 1.6 x 10-5 M sporofusarin caused a significant increase in the nonsedimenting activity of nearly all the enzymes; in a concentration of 1.6 x 10-3 M most of the enzymes of the lysosomal matrix (beta-glucuronidase, beta-galactosidase, aryl sulfatases A and B) were liberated almost completely into the supernatant, and nearly all the beta-glucosidase also was liberated. It is postulated that damage to the subcellular membranes is an important component of the toxic action of sporofusarin.

Animals↗
PubMed1993

Evidence for estrogen synthesis in adenomyotic tissues.

OBJECTIVE: To investigate steroidogenesis in eutopic and ectopic endometrial tissues in adenomyosis. STUDY DESIGN: Aromatase and estrone sulfatase activities were determined by anion-exchange resin column chromatography, thin-layer chromatography, and cocrystallization. The effects of danazol on the activity of these enzymes were also examined. Moreover, localization of aromatase in eutopic and ectopic endometrial tissues was immunohistochemically examined with antihuman placental aromatase cytochrome P-450 antibody. RESULTS: Aromatase and estrone sulfatase activities were detected in ectopic endometrium. The activity of these enzymes was significantly suppressed by danazol. In addition, aromatase was immunohistochemically detected in glandular cells of eutopic and ectopic endometrial tissues. CONCLUSIONS: The results suggest that estrogen is synthesized in the eutopic and ectopic endometrial tissues of women with uterine adenomyosis and that it may affect the growth of adenomyosis. Danazol suppressed synthesis of estrogen in vitro.

Adult↗
PubMed1995

Pregnancy outcome for women with very low levels of maternal serum unconjugated estriol on second-trimester screening.

OBJECTIVE: Our purpose was to assess pregnancy outcome for women whose second-trimester triple screen results revealed very low unconjugated estriol levels. STUDY DESIGN: Of 44,146 women screened for alpha-fetoprotein, human chorionic gonadotropin, and unconjugated estriol, a group of women with isolated very low unconjugated estriol levels were selected as the study group. Pregnancy outcome was obtained by contacting the referring physicians. RESULTS: During the study period 62 women had unconjugated estriol levels at or below the sensitivity of the assay. Fetal death was the most common explanation, accounting for 37.1% of cases. Six cases of early death followed uneventful prenatal visits. Eighteen women had unexplained very low unconjugated estriol levels. Of these 18, four cases of placental sulfatase deficiency were diagnosed. Sixteen of these 18 women gave birth to male infants (p = 0.03). CONCLUSION: A very low unconjugated estriol level in the second trimester is associated with increased risk for early death and placental sulfatase deficiency.

Adult↗
PubMed1985

Enzymatic analysis with chondrosulfatases of constituent disaccharides of sulfated chondroitin sulfate and dermatan sulfate isomers by high-performance liquid chromatography.

Various under-sulfated, monosulfated, and over-sulfated chondroitin sulfate and dermatan sulfate isomers were analyzed in terms of disaccharide units before or after desulfation with chondrosulfatases in addition to digestion with chondroitinases. The unsaturated disaccharides were separable by a high-performance liquid chromatography (HPLC) method using a resin made from a sulfonized styrene-divinylbenzene copolymer. The retention times of the parent sulfated unsaturated disaccharides and newly generated unsaturated mono- or nonsulfated disaccharides were reproducible. On desulfation of the parent sulfated unsaturated disaccharides with chondrosulfatases, almost all delta Di-S showed the same retention times as those of standard delta Di-S from known components. Following digestion of delta Di-diSB with chondro-4-sulfatase as well as delta Di-diSD or delta Di-diSG with chondro-6-sulfatase, three delta Di-monoS with the same retention time were detected with the HPLC method. These newly generated delta Di-monoS2 showed that the structure is N-acetyl-D-galactosamine, uronic acid 2-sulfate.

Chondroitin↗
PubMed1993

Estrogen productivity of endometrium and endometrial cancer tissue; influence of aromatase on proliferation of endometrial cancer cells.

Aromatase, estrone (E1) sulfatase and E1 sulfotransferase activities were examined in endometrium and endometrial cancer tissue preparations. Aromatase and E1 sulfatase activities in endometrial cancer tissues were found to be significantly higher than in normal endometrial tissues. However, E1 sulfotransferase activity did not differ between benign and malignant tissue. We also examined the effect of testosterone (T) on aromatase activity and tritiated thymidine uptake (DNA synthesis) in various cultured cervical or corpus endometrial cancer cell lines (OMC-4, HHUA, Ishikawa, HEC-59). The results demonstrated that only the HEC-59 cell line had high aromatase activity and increased its DNA synthesis in response to T. This increase of DNA synthesis by T was not suppressed by simultaneous addition of cyproterone acetate, but was by tamoxifen. These data suggest that in situ estrogen production in endometrial cancer tissue is biologically important and that aromatase in cancer cells may contribute partially to cell proliferation if androgen substrate is provided.

Aromatase↗
PubMed2006

Metabolism of pyrene by aquatic crustacean, Daphnia magna.

The aquatic crustacean Daphnia magna is an important species for ecotoxicological study, and is often used as a test organism for environmental risk assessment. However, the mechanism of xenobiotic metabolism by this species has not been studied in detail. In the present study, pyrene was used as model substance to investigate the mechanism of xenobiotic metabolism in D. magna. The results of 24-h exposure experiments showed that D. magna could metabolize pyrene and biotransform it into water-soluble metabolites. On the other hand, the metabolism of pyrene was significantly inhibited by SKF-525A as the cytochrome P450 (CYP) inhibitor. These observations indicated that oxidation by CYP participated in the biotransformation of pyrene by D. magna. We also identified the pyrene metabolites formed by D. magna by HPLC with an electrospray ionization triple quadrupole mass spectrometry detector (LC/ESIMS/MS) and de-conjugation by sulfatase, beta-glucuronidase, and beta-glucosidase. One of the metabolites was ionized in ESI negative mode and formed a dominant mass of m/z 297 (MS) with the product ion of m/z 217 (MS2). Furthermore, this metabolite formed 1-hydroxypyrene on treatment with sulfatase. This metabolite was considered to be a sulfate conjugate of oxidized pyrene (1-hydroxypyrenesulfate). Furthermore, we quantified the deconjugated 1-hydroxypyrene formed by the above enzyme treatment. It showed that 52% of the total metabolized pyrene was biotransformed into 1-hydroxypyrene-sulfate, and more than 73% was biotransformed into oxidized pyrene conjugate. These results indicated that CYP and several conjugation enzymes participate in its biotransformation, and sulfation is important in D. magna for metabolism and elimination of xenobiotics.

Animals↗
PubMed1975

Defective heparan sulfate metabolism in the Sanfilippo syndrome and assay of this defect in the assessment of the mucopolysaccharidoses patient.

The Sanifilippo syndrome is an inherited dementia caused by defective degradation of heparan sulfate. In the course of its catabolism the heparan sulfate polymer must be desulfated. Heparan sulfate sulfatase activity was demonstrated in homogenates of normal tissues and cultured skin fibroblasts, and in normal urine. This activity was found to be grossly depressed or absent in necropsy specimens of liver and spleen from two Sanfilippo patients. The heparan sulfate sulfatase activity was not demonstrable in urine from eleven, or cultured fibroblasts from four Sanfilippo patients. Activities of alpha-N-acetyl-glucosaminidase, the site of the metabolic defect in the Sanfilippo B variant were either normal or slightly elevated in the Sanfilippo tissues and cultured fibroblasts whereas the mean level in the urine of our Sanfilippo patients was about one-third of that encountered in control urines.

Acetylglucosaminidase↗