Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: EC:3.1.6.1 (sulfatase)
 3,205 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The first case of successful bone marrow transplantation (BMT) in a patient with I-cell disease is reported. A 8-month-old girl with I-cell disease (N-acetylglucosaminylphosphotransferase deficiency) has had successful reconstitution with bone marrow from her HLA-MLC-matched brother who has heterozygous level of the transferase activity. The following biochemical and clinical improvements have occurred: the transferase in peripheral lymphocytes increased to donor's level, and lymphocytic alpha-neuraminidase, beta-galactosidase and alpha-mannosidase increased to normal levels. Plasma acid hydrolase activities, which had been 10 to 60 times higher in the patient than normal control levels, have slowly but steadily decreased from one month after the graft. Such decreases were observed in the activities of alpha-mannosidase, N-acetyl-beta-glucosaminidase, alpha-fucosidase, arylsulfatase A and acidic beta-galactosidase. There was also a marked decrease of vacuolated peripheral lymphocyte after the BMT. Three-months after the engraftment, hepatomegaly gradually decreased in size, corneal clouding has not progressed, and tight skin seems to have improved.
Tohoku J Exp Med 1986 Sep
PMID:Biochemical improvement after treatment by bone marrow transplantation in I-cell disease. 302 24

The physiological relevance of the ability of beta-N-acetylhexosaminidase A to liberate N-acetylglucosamine 6-sulfate from polymeric keratan sulfate was investigated. Upon intravenous injection into rats of [35S]sulfate-labeled proteokeratan sulfate up to 25% of the radioactivity excreted with the urine were identified as N-acetyl-glucosamine 6-sulfate. Within 24 h, however, excretion of inorganic sulfate rose at the expense of the sulfated monosaccharide. Upon incubation in vitro of liver lysosomes from rats treated with proteokeratan sulfate, inorganic sulfate and minor amounts of sulfated monosaccharide were found in the incubation fluid. Cultured rat peritoneal macrophages ingested proteokeratan sulfate with a clearance rate of 6-9 micrograms X h-1 X mg cell protein-1 and degraded it rapidly. Inorganic sulfate but not N-acetylglucosamine 6-sulfate was delivered to the culture medium. During a chase period the amount of intracellular N-acetylglucosamine 6-sulfate fell, and a corresponding amount of sulfate could be found extracellularly. Significant amount of N-acetylglucosamine 6-sulfate were only found in the culture medium when the cells were challenged with zymosan. These results suggest that N-acetylglucosamine 6-sulfate is a physiological intermediate during the degradation of keratan sulfate, but is usually hydrolyzed intralysosomally by N-acetylglucosamine-6-sulfate sulfatase. Genetic deficiency of the sulfatase in humans therefore results in excessive excretion of the sulfated amino sugar but not of keratan sulfate.
Eur J Biochem 1985 Sep 16
PMID:Intralysosomal formation and metabolic fate of N-acetylglucosamine 6-sulfate from keratan sulfate. 316 30

A liquid chromatographic (LC) method is described for the determination of the plant estrogens diadzein, formononetin, and coumestrol and the estrogenically active metabolite equol in bovine blood plasma and urine. The blood and urine samples are incubated overnight with and without beta-glucuronidase/sulfatase for analysis of both free and conjugated forms of estrogens. Samples are applied to Extrelut columns, extracted with ethyl acetate, and evaporated to dryness. Residues from urine samples are dissolved in methanol, diluted with water, acidified with HCl, and purified by injection through a Sep-Pak C18 cartridge. This eluate is used for LC analysis. Residues from blood samples are dissolved in benzene-petroleum ether (1 + 1), extracted with ammonium hydroxide, acidified with glacial acetic acid, and extracted with ethyl acetate. The ethyl acetate extract is evaporated, dissolved in 80% methanol, injected onto a LC reverse-phase column, and separated in a linear gradient system between 40 and 80% methanol in phosphate buffer. Quantitation is performed by means of UV and fluorescence responses. The method was sensitive enough to determine 0.4 ng/mL of daidzein and formononetin and 0.1 and 13 ng/mL of coumestrol and equol, respectively, in blood, and 130, 80, and 7 ng/mL of daidzein, formononetin, and coumestrol, respectively, and 4 micrograms/mL of equol in urine. The applicability of the method was checked by the determination of total and free plant estrogens in blood samples from a dairy cow fed a normal diet.
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PMID:Liquid chromatographic determination of the estrogens daidzein, formononetin, coumestrol, and equol in bovine blood plasma and urine. 323 13

The biochemical analysis of gingival crevicular fluid (GCF) may offer a sensitive means of determining periodontal disease activity, including the transition of gingivitis to periodontitis. To continue our evaluation of the relationship between clinical and GCF parameters, 552 sites with shallow to intermediate (2.0-5.0 mm) probing depths (PD) were examined. The data were collected at baseline from 33 periodontitis patients participating in a longitudinal trial examining the relationship of changes in GCF biochemistry to attachment loss. Mesiobuccal sites were scored for dichotomous measures of bleeding on probing, gingival redness, suppuration, and plaque accumulation. In addition, GCF was collected using filter paper strips inserted into the sulcus for 30 seconds, eluted in buffer and assayed for activity of the enzymes beta-glucuronidase (BG), arylsulfatase (AS), and lactate dehydrogenase (LDH), markers for ground substance-degradation and cellular necrosis, respectively. Clinical and GCF parameters were evaluated by increasing PD. Plaque accumulation and bleeding on probing increased with increasing PD, although there was considerable overlap across groups. Suppuration was present in only a very small number of sites and the proportion of sites displaying gingival redness was not related to PD. GCF volume was grouped in 0.25-microliter increments, revealing a progressive shift with increasing PD toward a normal distribution around the median range of 0.51 to 0.75 microliter at 5.0 mm. Mean enzyme activities of BG, and to a lesser extent AS and LDH increased sharply from 2.0 to 3.0 mm, were relatively stable from 3.5 to 4.5 mm, and were significantly higher in 5.0 mm than 4.5 mm sites.(ABSTRACT TRUNCATED AT 250 WORDS)
J Periodontol 1987 Sep
PMID:Lysosomal and cytoplasmic enzyme activity, crevicular fluid volume, and clinical parameters characterizing gingival sites with shallow to intermediate probing depths. 330 52

It is known that prostaglandin (PG)E2 and PGI2 can contribute to the ripening of the uterine cervix. To study the PG biosynthesis in cervical tissue, 14C-arachidonic acid was used to incubate the preparation of human cervical tissue obtained from pregnant women at delivery and non pregnant women at hysterectomy. Labeled PGE2 and 6-keto-PGF1 alpha (6PG), a stable metabolite of PGI2 were isolated on TLC, and the enzymatic activity was calculated from the formation of PGE2 and 6PG from arachidonic acid. The capacity to metabolize arachidonic acid to PGE2 and 6PG in cervical tissue obtained from pregnant women was 6 times higher than that from non pregnant women. Low enzymatic activity in the formation of PGE2 and 6PG were observed in cervical tissues from the patients with placental sulfatase deficiency and preterm delivery which were known to have a low estrogen environment. On the other hand, DHA-S administration to patients increased the formation of both PGE2 and 6PG. These results demonstrate that human cervical tissue possesses the ability to synthesize PGE2 and PGI2, and enzymatic activity increased during pregnancy, and was further enhanced by the administration of DHA-S. The results suggest that the steroids in the fetoplacental unit may be involved in the mechanism controlling the formation of PGs in the cervical tissue which lead the cervix to ripen at term.
Nihon Sanka Fujinka Gakkai Zasshi 1987 Sep
PMID:[Biosynthesis of prostaglandin in human cervical tissue]. 331 40

A sensitive and specific RIA has been developed to measure thyronine (To) in urine. The RIA used an anti-To antibody obtained from a rabbit immunized with a L-To-human serum albumin conjugate and [3H]To as the radioligand. The acetic acid analog of To (ToAc), that is the diphenyl structure with an acetic acid side-chain, cross-reacted strongly with the antibody. Relative to To, it cross-reacted 160% in phosphate-buffered saline, pH 7.4, and 100% in 0.075 mol/L barbital buffer, pH 8.6, containing sodium salicylate (final concentration, 8 mg/mL). The latter conditions were employed for the RIA, and the results reported thus reflect the presence of To and/or ToAc. 3-Monoiodothyronine, 3'-monoiodothyronine, 3',5'-diiodothyronine, and 3,5-diiodothyronine cross-reacted with the anti-To antibody 1.9%, 1.7%, 0.3%, and 0.2%, respectively; the cross-reactivity of other To derivatives and tyrosine and its derivatives was less than 0.05%. Urinary To and/or ToAc excretion in 12 normal subjects averaged 16 +/- 2 (+/- SE) micrograms/day (59 +/- 9 nmol/day) or 14 +/- 2 micrograms/g creatinine (5.9 +/- 0.6 nmol/mmol creatinine). Treatment of urine from normal subjects with beta-glucuronidase or sulfatase did not significantly alter the To content. Column and thin layer chromatographic studies revealed that 83% and 61%, respectively (range, 37-100%), of urinary To immunoreactivity was attributable to ToAc. The mean daily excretion of To in 20 patients with nonthyroidal illness [NTI; 22 +/- 4 micrograms/day (82 +/- 17 nmol/day)] was similar to that in normal subjects, but was elevated when expressed as nanomoles per mmol creatinine (20 +/- 2; P less than 0.001), because creatinine excretion was reduced in the NTI patients. The mean daily urinary To excretion in 13 patients with hyperthyroidism due to Graves' disease was slightly elevated [29 +/- 6 micrograms/day (108 +/- 21 nmol/day); P less than 0.1], but was clearly elevated when expressed as nanomoles per mmol creatinine (37 +/- 8; P less than 0.001), again because creatinine excretion was reduced in these patients. The mean urinary To excretion was subnormal in 13 patients with hypothyroidism and was significantly (P less than 0.005) less than that in the NTI patients regardless of the manner in which the results were expressed. Analysis of pronase hydrolysates of thyroid glands obtained at autopsy from euthyroid patients suggested that the To content of the thyroid approximates only 1.2% that of T4, supporting the thesis that prior iodination of tyrosine is critical for the coupling process in the thyroid.(ABSTRACT TRUNCATED AT 400 WORDS)
J Clin Endocrinol Metab 1988 Sep
PMID:A radioimmunoassay for measurement of thyronine and its acetic acid analog in urine. 341 Sep 34

Sulfokinase, sulfatase, 17 beta-HSD, 20 alpha-HSD, 3 beta-HSD and 5 alpha-reductase activity and steroid concentrations including estradiol, estrone, estrone-sulfate, progesterone, 20 alpha-dihydroprogesterone, DHA and DHA-sulfate in endometrial tissue were examined in order to study the changes in steroid metabolism in relation to the menstrual cycle in the human endometrium. Thirty-one (14) proliferative and 17 secretory) endometrial tissue samples were obtained from women who underwent hysterectomy. Low enzymatic activity of sulfokinase, sulfatase and 17 beta-HSD activity were observed in the proliferative phase (0.25, 8.5, 3.1 nmole/mg protein/h). A pronounced increase in enzymatic activity was observed in the early secretory phase and activity gradually decreased toward the mid and late secretory phase. On the other hand, 20 alpha-HSD and 3 beta-HSD activity did not change during the cycle. 5 alpha-reductase activity was not detectable under the conditions used. The concentration of progesterone in the secretory phase was significantly higher than that in the proliferative phase. The concentration of estradiol in the proliferative phase was significantly higher than that in the secretory phase. There was no significant change in the concentration of estrone, estronesulfate, 20 alpha-dihydroprogesterone, DHA or DHA-sulfate during the cycle. The relationship between the steroid concentration and the enzymatic activity was discussed. The results suggested an active role of the endometrium in controlling the biological effect of steroids.
Nihon Sanka Fujinka Gakkai Zasshi 1987 Sep
PMID:[Changes in steroid enzyme activity in the human endometrium during the menstrual cycle]. 350 Feb 44

Using a reproducible approach to collection, processing and analysis of gingival crevicular fluid (GCF), this study examined 284 fluid samples from individual crevicular sites for the presence of the enzymes lactate dehydrogenase (LDH), B-glucuronidase (BG) and arylsulfatase (AS). 88 of the sites were from periodontally healthy individuals (probing depth 1-3 mm), while 98 sites from patients with periodontitis were examined before and 2 weeks after scaling and root planing (probing depths 1-3 mm, 4-6 mm and 7-10 mm). This study demonstrated the sensitivity of the enzyme assays. When GCF was collected with a 30-s insertion of the filter strip, 90% of the sites from the control subjects demonstrated LDH activity, 85% demonstrated BG activity and 73% demonstrated AS activity. For the 1-3 mm sites from the patients with periodontitis, 100% of sites from which fluid was collected demonstrated LDH and BG activity, and 90% of sites had AS activity before therapy. After therapy, 100% of sites demonstrated LDH activity, 90% had BG activity and 83% had AS activity. All sites in the 4-6 mm and 7-10 mm categories demonstrated activity of all 3 enzymes. The data were analyzed in terms of enzyme activity/30-s sample and as concentration of enzyme in a standard volume of GCF. Enzyme activity/30-s sample was a different and possibly more sensitive indicator of periodontal pathology than standard clinical parameters. There was a disassociation between clinical parameters and the data for enzyme analysis when it was reported as concentration.
J Clin Periodontol 1986 Sep
PMID:Enzyme activity in human gingival crevicular fluid: considerations in data reporting based on analysis of individual crevicular sites. 353 4

The disposition of 2,3,4,7,8-pentachlorodibenzofuran (PeCDF), a highly toxic environmental contaminant which accumulates in human tissues, was examined in the male Fischer rat after iv and oral exposure. Greater than 70% of an oral dose of 0.1, 0.5, or 1.0 mumol PeCDF/kg body wt was absorbed by the gastrointestinal system. After either oral or iv administration of 0.1 mumol/kg, the dibenzofuran was rapidly removed from the blood and accumulated in the liver and adipose tissue and to a lesser extent in the skin and muscle. Three days after administration, 70% of the iv dose of PeCDF was found in the liver, 7% in the fat, 1% in the skin, and 0.5% in the muscle. Route of exposure had little effect on tissue distribution. TLC analyses indicated that greater than 99% of the [14C]-PeCDF-derived radioactivity which had accumulated in the liver and adipose tissue was unmetabolized PeCDF which was eliminated very slowly (t1/2 = 193 and 69 days, respectively). The whole body half-life calculated from the daily fecal excretion rate was approximately 64 days. Excretion occurred primarily via the feces. No radioactivity was detected in expired air and less than 0.02% was detected in the urine. TLC analysis of fecal extracts indicated greater than 90% of the [14C]PeCDF-derived radioactivity in the feces was polar metabolites of the parent compound. Pretreatment with 500 micrograms PeCDF/kg body wt caused biliary excretion to nearly double. Treatment of bile with beta-glucuronidase or arylsulfatase had little effect on the chromatographic profile. Therefore, PeCDF was readily absorbed from the gastrointestinal tract, concentrated primarily in the liver, and was slowly eliminated from the body as polar metabolites. The long half-life and high body burden of PeCDF suggest that the toxicity of this chemical may be enhanced due to bioaccumulation upon chronic low-level exposure.
Toxicol Appl Pharmacol 1987 Sep 15
PMID:Disposition and excretion of 2,3,4,7,8-pentachlorodibenzofuran in the rat. 362

The influence of the peptide hormone relaxin on the glycosaminoglycan (GAG) metabolism was investigated in the pubic ligament of the symphysis pubis and in serum of the virgin mouse. Fresh weight DNA and GAG content per 1 ligament is significantly increased, the level of water soluble protein is not affected. A shift in the electrophoretic GAG pattern by an increasing amount of hyaluronic acid and a decreasing amount of chondroitin sulfate and dermatan sulfate can be observed. Concerning GAG-splitting enzymes (N-acetylglucosaminidase, arylsulfatase, beta-glucuronidase) the N-acetylglucosaminidase reveals a significant increase of its activity in the interpubic ligament and in the serum. The data demonstrate that relaxin treatment induces some changes in the GAG metabolism.
Horm Metab Res 1987 Sep
PMID:Effects of the hormone relaxin on the metabolism of the glycosaminoglycans in the mouse symphysis pubis. 369 38


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