Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound   Target Concepts: Gene/Protein Disease Symptom Drug Enzyme Compound

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Query: EC:3.1.3.1 (alkaline phosphatase)
47,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The paucity of the intrahepatic bile ducts, also known as ductopenia, is a well recognized disorder in pediatric patients. Recently, however, a similar disorder has been reported in adults and termed idiopathic adulthood ductopenia (IAD). We describe a 30-year-old patient with a 15 year history of episodes of jaundice. During icteric episodes, serum levels of bilirubin and alkaline phosphatase were markedly elevated. Between attacks, totalling more than 30, the patient was asymptomatic, but bilirubin and alkaline phosphatase levels were mildly elevated. No neonatal jaundice was present in the patient's history. PBC, PSC and drug-induced cholestasis were excluded. Two needle biopsies of the liver, taken within a 13 year interval, were available. The lobular architecture appeared progressively disturbed by porto-centro-portal bridging septa. In both biopsies, a destructive cholangitis was found. In the last biopsy, the majority of the septal and interlobular ducts appeared severely damaged and, in three out of seven portal tracts, the interlobular bile duct had disappeared. In the parenchyma, the main feature was a severe mainly canalicular bilirubinostasis. The patient described illustrates that IAD may have a clinical picture indistinguishable from benign recurrent intrahepatic cholestasis. The etiology of the disease, in this as in other patients, remains unknown.
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PMID:Idiopathic adulthood ductopenia presenting with chronic recurrent cholestasis. A case report. 200 71

S-Adenosylmethionine (SAMe) proved to be effective in antagonizing bile secretion impairment induced by a wide range of hepatotoxins, including ethynylestradiol, taurolithocholate, chlorpromazine and alpha-naphthyl-isothiocyanate. The anticholestatic activity of SAMe may result from its role in the intermediate metabolism as this molecule is involved in transmethylation and transsulfuration reactions. Clinical experience, carried-out on more than 1,000 cholestatic patients, supports preclinical data. In particular, controlled clinical trials have documented that intravenous SAMe (800 mg/day) induced a significant decrease of biochemical parameters of cholestasis (serum total and conjugated bilirubin, serum total bile salts, and aminotransferases), as well as a significant improvement of pruritus in women with ICP compared with placebo. In addition, other studies provided the evidence that both parenteral (800 mg/day) and oral SAMe (1600 mg/day) significantly improves subjective (pruritus, fatigue, and general discomfort) and objective (serum total and conjugated bilirubin, and serum alkaline phosphatase) parameters of cholestasis in patients with intrahepatic cholestasis complicating chronic liver diseases compared with placebo. In all these trials, SAMe treatment has been well tolerated at the same extent as placebo. In conclusion, experimental and clinical investigations indicate that SAMe represents an effective and safe approach to the management of intrahepatic cholestasis.
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PMID:A review of the studies on the clinical use of S-adenosylmethionine (SAMe) for the symptomatic treatment of intrahepatic cholestasis. 217 53

The distribution of trace elements in kidney cortex and medulla and in their subcellular fractions was studied by inductively coupled plasma mass spectrometry (ICP-MS). This technology allows the analysis of almost all the elements of the periodic table, along with their isotopes, in the same experiment. Acid digestion of the tissue (samples) was required before ICP-MS elemental analysis. Mineralization in 8 N nitric acid for 2 min in a Parr microwave acid digestion bomb, inside a regular microwave oven working at medium power, gave good sensitivity and reproducible results. Trace element determinations could be precisely performed, despite the presence of a considerable amount of organic matter. Cortex/plasma and medulla/plasma inorganic ratios were taken as indicators of the inorganic bioaccumulation. The highest enrichment factors of elements found in the cortex were Mn greater than Co greater than Tl greater than Zn greater than Rb greater than Mo greater than Cu and the highest element enrichments of the medulla were Tl greater than Mn greater than Co greater than Rb greater than Zn greater than Bi greater than Mo greater than Ni greater than Cu. Subcellular fragmentation of the kidney cortex and medulla indicated that the majority of the trace elements was located in the cytosolic fraction. The membrane fraction of the medulla contained significant amounts of Hg and Al: 120.7 and 32.0 mg/g protein, respectively. Cu and Zn were the most abundant trace elements found in the brush border membrane from the cortex: 120.7 and 68.2 mg/g protein, respectively. These quantities are largely in excess of what is bound, for example, to alkaline phosphatase.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Complete analysis of the trace elements of the kidney. 227 3

Recent findings that retinoic acid (RA) induces terminal granulocytic differentiation of the human promyelocytic leukemia cell line HL-60 in vitro and blast cell maturation in patients suffering from acute non-lymphocytic leukemia (ANLL) prompted an investigation on the ability of this agent to induce terminal maturation in blast cells from ANLL patients in vitro. We tested the ability of RA at 3 x 10(-6) M, 3 x 10(-7) M and 3 x 10(8-) M concentrations to induce differentiation in blastoid cells from 16 patients with ANLL using cytochemical and cytologic parameters, in addition to cytofluorometric methods. Leukemic cells in primary culture from all the patients underwent cytochemical and biochemical changes after treatment with RA. However, the extent of differentiation-positive cell clones (D+ clones) varied from patient to patient. Morphologic maturation was observed in a significant number of bone marrow samples. Leukocyte alkaline phosphatase and NBT reduction ability of cells, which are biochemical markers of granulocytic differentiation, were also significantly increased with a simultaneous decrease in DNA and RNA synthesis (which was estimated using a Phywe ICP-11 impulse flow cytometer).
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PMID:Multiparametric evaluation of retinoic acid-induced terminal differentiation of blastoid cells from acute non-lymphocytic leukemia patients in vitro. 248 50

Mild abnormalities of liver function tests are frequently seen in pregnancy but return to normal after delivery. A raised serum alkaline phosphatase is common, along with a decline in the serum albumin, but the aminotransferases remain within normal limits. The physician must interpret abnormal liver function tests in pregnancy with these changes in mind, but most liver diseases in pregnancy result in more marked alterations. Viral hepatitis is the most common cause of jaundice in pregnancy, and the maternal prognosis is generally good. Perinatal transmission of hepatitis B virus is likely when the mother is positive for HBsAg. Concurrent administration of hepatitis B vaccine and HBIG to the infant has an efficacy of 90 per cent in preventing transmission to the infant. ICP is the second most common cause of jaundice in pregnancy. The condition is generally benign, although maternal and fetal mortality occasionally result, probably due to premature delivery and the bleeding tendency of cholestatic patients. Vitamin K administration may correct the coagulopathy, and cholestyramine is effective in controlling pruritus. AFLP is rare but carries a high mortality rate for both the mother and the fetus. Early diagnosis, correction of the coagulopathy, and prompt delivery may improve the outcome significantly. Patients with cirrhosis have reduced fertility, and in those who become pregnant, fetal loss is high. The effect of pregnancy or hepatocellular function is variable, but, when evidence of liver failure is present in the first trimester, termination should be considered. Variceal size and the risk of bleeding may be assessed by endoscopy. Pregnant cirrhotic patients with large esophageal varices and a history of bleeding can undergo shunt surgery. Conservative management may be appropriate for patients with small varices and no history of bleeding.
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PMID:Liver diseases in pregnancy. 405 85

The diagnosis of benign recurrent intrahepatic cholestasis was made in a 40 year old female. Seven episodes of jaundice persisting up to 6 months in lengths lead to 3 laparotomies, numerous peritoneoscopies and needle biopsies of the liver. Elevation of conjugated serum bilirubin and alkaline phosphatase levels and histopathological confirmation of intrahepatic cholestasis without cholangitis were the main characteristics during the episodes of cholestatic jaundice. However, hepatic histology and liver function were normal in remission periods. Drug induced cholestasis was ruled out by careful history. A sister also suffered from 4 episodes of intrahepatic cholestasis. The etiology of the disease is probably of a genetic origin. The pathogenesis remains unclear. There is no specific treatment to prevent or shorten the occurrence of cholestatic episodes. The patients are advised to avoid pregnancy and oral contraceptives, since both will induce icteric phases.
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PMID:[Benign recurring intrahepatic cholestasis--a case report]. 646 22

Benign recurrent intrahepatic cholestasis (BRIC or Summerskill-Walshe-Tygstrup-syndrome) is a rare autosomal recessive form of liver disease, which usually becomes manifest in childhood. Characteristic are recurrent episodes of jaundice and itching of different duration. Number and duration of episodic attack and asymptomatic period develop individually. For diagnosis of BRIC following criteria are proposed: At least three episodes of severe jaundice and pruritus with biochemical evidence of cholestasis, normal intra-and extrahepatic bile ducts on cholangiography, absence of a factor known to produce intrahepatic cholestasis and symptom-free intervals of several months or years. Often the diagnosis of BRIC is made very late and patients have to suffer invasive investigations (explorative laparotomy). Because of the unknown pathophysiological mechanism there is no specific treatment. We report on a 53-year-old patient with jaundice, severe pruritus, vomiting, loss of hair and weight, extreme sleeplessness and intractable cough. At the onset of the attack an increase of serum bilirubin concentration and serum alkaline phosphatase was observed, whereas aspartate and alanine aminotransferase and gamma-glutamyltransferase were normal. Histological findings of liver biopsy revealed accumulation of bile plugs in bile canaliculi. The long-term follow-up of our patient confirms that the prognosis is good.
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PMID:[Follow-up of benign recurrent intrahepatic cholestasis (Summerskill-Walshe-Tygstrup syndrome) over 46 years]. 965 5

Benign recurrent intrahepatic cholestasis is a rare autosomal recessive disorder characterized by repeated episodes of intense pruritus, profound elevations in serum alkaline phosphatase and bilirubin, with normal or nearly normal values for serum gamma-glutamyl transferase. Attack lasts from several weeks to months and resolve spontaneously. Between attacks patients remain asymptomatic for months to years. The disorder does not lead to progressive liver injury and is not fatal. Genetic studies have demonstrated that the disorder is the result of a mutation in ATP8BI, a gene that codes for the FIC1 (familial intrahepatic cholestasis) protein, which is also affected in other forms of familial intrahepatic cholestasis. It is believed this protein plays a role in bile acid secretion, in aminophospholid transport, and in maintaining fluidity of the cell membrane. Therapy is supportive and aimed at relieving pruritus and other complications of severe cholestasis until the episode resolves spontaneously.
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PMID:Benign recurrent intrahepatic cholestasis. 1506 97

DNA is a universal analyte found in almost every organism. It is the code that dictates our genetic make-up and it provides a vast library of information. DNA sequences can indicate genetic modification of foodstuffs, how we may metabolise pharmaceuticals and the likelihood of suffering particular diseases. The basis for many of these genetic tests would benefit greatly from procedures that can accurately quantitate DNA in an absolute manner. This would then provide a sound and universally consistent foundation for regulatory and diagnostic decision making. This work compares two different enzymatic digestion systems as precursor steps to high accuracy isotope dilution mass spectrometry (IDMS) quantitation of a 20mer oligonucleotide. In the first approach, snake venom phosphodiesterase (SVP) digests the oligonucleotide to its constituent deoxynucleotides (dNMPs), followed by liquid chromatography-IDMS (LC-IDMS) quantitation. The second enzyme digestion approach used a combination of snake venom phosphodiesterase and shrimp alkaline phosphatase (SAP) which reduces the oligonucleotide to its constituent deoxynucleosides (dNs). This was then followed by an alternative LC separation and equivalent IDMS measurements. Total phosphorous content of the 20mer oligonucleotide was measured by inductively coupled plasma optical emission spectroscopy (ICP-OES). This provided independent data for comparison with the two enzyme digestion-IDMS based procedures. The most appropriate method of quantitation was found to be the combined SVP and SAP digestion. This approach negates the need to consider and/or account for the lack of a 5' terminal phosphate residue. It also enables the use of positive ion mass spectrometry which simplifies the chromatographic requirements. Based on the exact matched IDMS of the adenine deoxynucleoside, the concentration of the original 20mer oligonucleotide was found to be 110+/-9 microg g(-1). This showed good agreement with the ICP-OES data based on the measurement of phosphorus which gave an equivalent value for the original 20mer oligonucleotide of 108+/-5microg g(-1) (uncertainties at the 95% confidence interval). It is intended that this high accuracy methodology should be used to produce high calibre reference standards. These, in turn, could then be used to underpin the quality and consistency of routine measurements involving a variety of more commonly encountered methodologies. It should be noted that the IDMS procedures are equally applicable to both sequenced and non-sequenced oligonucleotide materials.
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PMID:A comparison of enzymatic digestion for the quantitation of an oligonucleotide by liquid chromatography-isotope dilution mass spectrometry. 1568 82

Alpha-naphthyl isothiocyanate (ANIT) is a hepatotoxicant that produces acute intrahepatic cholestasis in rodents. Farnesoid X receptor (FXR) and pregnane X receptor (PXR) are two major bile acid sensors in liver. The purpose of this study was to characterize the regulation of hepatic transporters by FXR and PXR during ANIT-induced liver injury. Wild-type, FXR-null, and PXR-null mice were administered ANIT (75 mg/kg, po) and evaluated 48 h later for hepatotoxicity and messenger RNA (mRNA) expression of basolateral uptake (sodium taurocholate-cotransporting polypeptide, organic anion transporting polypeptide [Oatp] 1a1, Oatp1a4, Oatp1b2) and efflux transporters (organic solute transporter [Ost] alpha, Ostbeta, multidrug resistance-associated protein [Mrp] 3, Mrp4), as well as canalicular transporters (bile salt export pump [Bsep], Mrp2, multidrug resistance protein 2 [Mdr2], ATPase, class I, type 8B, member 1 [Atp8b1]). Livers from wild-type and PXR-null mice had comparable multifocal necrosis 48 h after ANIT. However, ANIT-treated FXR-null mice have fewer and smaller necrotic foci than wild-type mice but had scattered single-cell hepatocyte necrosis throughout the liver. Serum alanine transaminase, alkaline phosphatase (ALP), and direct bilirubin were increased in all genotypes, with higher ALP levels in FXR-null mice. Serum and liver unconjugated bile acids were higher in ANIT-treated FXR-null mice than the other two genotypes. ANIT induced mRNA expression of Mdr2, Bsep, and Atp8b1 in wild-type and PXR-null mice but failed to upregulate these genes in FXR-null mice. mRNA expression of uptake transporters declined in livers of all genotypes following ANIT treatment. ANIT increased Ostbeta and Mrp3 mRNA in livers of wild-type and PXR-null mice but did not alter Ostbeta mRNA in FXR-null mice. In conclusion, FXR deficiency enhances susceptibility of mice to ANIT-induced liver injury, likely a result of impaired induction of hepatobiliary efflux transporters and subsequent hepatic accumulation of unconjugated bile acids.
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PMID:Compensatory induction of liver efflux transporters in response to ANIT-induced liver injury is impaired in FXR-null mice. 1940 37


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