Abstract：
:Fucosidosis is an inherited lysosomal storage disease due to a deficiency of alpha-L-fucosidase activity. Exponentially growing lymphoid cell cultures from a fucosidosis patient (JH) had 16-fold lower extracellular alpha-L-fucosidase protein and 72-fold lower intracellular alpha-L-fucosidase protein with negligible catalytic activity as compared with the mean of 19 control cultures. The percentage of total alpha-L-fucosidase protein released extracellularly by JH cells was 71% as compared with 35% +/- 9% for control cells. During a 1.5 h pulse with 35S-methionine, alpha-L-fucosidase was synthesized by JH cells as an intracellular doublet with Mr of 58,000 and 56,000 and by control cells as an intracellular form with Mr = 58,000. During a subsequent 21 h chase with unlabeled methionine, JH alpha-L-fucosidase was entirely secreted. In contrast, only 25%-30% of control enzyme was secreted with the remainder retained intracellularly. Thus, JH lymphoid cells synthesized a reduced amount of alpha-L-fucosidase that was catalytically inefficient and was hypersecreted. Treatment of JH alpha-L-fucosidase with N-glycanase produced polypeptide chains with Mr of 52,000 and 54,000. Previously, treatment of control alpha-L-fucosidase with N-glycancase produced a single polypeptide chain with Mr of 52,000 (Biochem Genet 1988; 26: 401-20). The doublet polypeptide chains of alpha-L-fucosidase in JH cultures may represent expression of two distinct allelic forms of mutant alpha-L-fucosidase.
journal_name
Clin Biochemjournal_title
Clinical biochemistryauthors
DiCioccio RA,Gordon BAdoi
10.1016/0009-9120(91)80018-xsubject
Has Abstractpub_date
1991-06-01 00:00:00pages
265-70issue
3eissn
0009-9120issn
1873-2933pii
0009-9120(91)80018-Xjournal_volume
24pub_type
杂志文章abstract：:Plasma levels of dehydroepiandrosterone sulfate, dehydroepiandrosterone and delta 5-androstenediol were measured in several women throughout pregnancy. The levels of both dehydroepiandrosterone and its sulfate decreased while those of delta 5-androstenediol did not. The levels of binding of the latter steroid to plasm...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/s0009-9120(80)90795-x
更新日期：1980-06-01 00:00:00
abstract：:Generally acid phosphatase (ACP) assay is used for testing cases of alleged rape. Comparison of three different chemical methods for Acid Phosphatase (Andersch's method with p-nitrophenyl-phosphate substrate and tartrate inhibitor (A-Tart), Roy's method with thymolphthalein phosphate substrate (R-TMP), and Babson's me...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/s0009-9120(77)92784-9
更新日期：1977-10-01 00:00:00
abstract：OBJECTIVE:To quantitatively summarize the association between tumour necrosis factor alpha (TNF-alpha) gene polymorphisms and Graves' disease. DESIGN AND METHODS:Relevant studies were identified from the following electronic databases: Cochrane Library, MEDLINE, EMBASE and Chinese Bio-medicine Database. A meta-analysi...
journal_title：Clinical biochemistry
pub_type： 杂志文章,meta分析
doi：10.1016/j.clinbiochem.2008.04.014
更新日期：2008-07-01 00:00:00
abstract：:A high pressure liquid chromatographic method for the determination of hydrochlorothiazide in serum has been elaborated and verified in a blood level study in normal individuals given a single oral dose of 50 mg hydrochlorothiazide. Based on 5 ml of serum, the limit of detection of the method is 2 ng/ml. ...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/s0009-9120(78)90434-4
更新日期：1978-08-01 00:00:00
abstract：OBJECTIVES:Microarray analysis of gene expression profiles of blood leukocytes has many potential clinical and research applications. DESIGN AND METHODS:We used the PAXgene Blood RNA System to prepare RNA from the whole blood of normal volunteers using two incubation times followed by gene expression profiling using t...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2004.03.013
更新日期：2004-09-01 00:00:00
abstract：:We describe a simple direct extraction method for the gas-liquid chromatography determination of serum valproic acid. The working range for the assay is 2-180 mg/L and our within-run precision was 5.8 and 4.3% at the 40 and 90 mg/L concentrations respectively. Hemolyzed and lipemic sera as well as samples from patient...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/s0009-9120(81)90772-4
更新日期：1981-04-01 00:00:00
abstract：OBJECTIVE:This study aimed at evaluating Elecsys free triiodothyronine (FT3) assay performed on an Elecsys 2010 system, while paying special attention to age relationship in euthyroid subjects. DESIGN AND METHODS:FT3 levels were measured in 149 euthyroid control subjects aged between 2 and 92 years old, 33 hyperthyroi...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/s0009-9120(98)00024-1
更新日期：1998-07-01 00:00:00
abstract：:We report a case of metastatic adenocarcinoma of the prostate with widespread bony and hepatic involvement having extremely high BB isoenzyme of creatine kinase in serum (2405 U/liter). The serum LD5/LD1 ratio is greater than one, with total LD activity more than twenty-five fold the normal value. The homogenate of pr...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/s0009-9120(80)90502-0
更新日期：1980-02-01 00:00:00
abstract：OBJECTIVES:This study examines the redox status of drug-resistant epileptic patients and how it is modified after surgical treatment. DESIGN AND METHODS:The activity of antioxidant enzymes (superoxide dismutase, catalase and glutathione peroxidase), and markers of damage to biomolecules (malondialdehyde and advanced o...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2006.11.019
更新日期：2007-03-01 00:00:00
abstract：OBJECTIVES:To determine the serum ratio of holo-ceruloplasmin to total ceruloplasmin in patients with Wilson's disease (WD), we identified apo- and holo-forms of serum ceruloplasmin with native-PAGE electrophoresis. DESIGN AND METHODS:Serum obtained from nine patients with Wilson's disease was subjected to native-PAGE...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2004.09.008
更新日期：2005-01-01 00:00:00
abstract：:Post-transplant ischemic acute kidney injury (AKI), secondary to ischemia reperfusion injury (IRI), is a major problem influencing on the short and long term graft and patient survival. Many molecular and cellular modifications are observed during IRI, for example, tissue damage result production of reactive oxygen sp...
journal_title：Clinical biochemistry
pub_type： 杂志文章,评审
doi：10.1016/j.clinbiochem.2018.03.022
更新日期：2018-05-01 00:00:00
abstract：OBJECTIVE:The immune-inflammatory system has been implicated in the pathogenesis of diabetic nephropathy; however, many of the mechanisms involved remain unclear. Chitotriosidase enzyme is an active human chitinase and a major protein product of activated macrophages. Although playing an important role in innate and ac...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2015.11.009
更新日期：2016-04-01 00:00:00
abstract：:The ES 300 system, a fully automated multichannel immunoassay analyzer, was evaluated simultaneously for 9 weeks in four major centers. Precision, accuracy, carryover, comparison to in-house methods, and interferences were assessed for the following 17 tests: T4, T3, FT4, TSH, TBK, TBG, LH, FSH, prolactin, HCG, digoxi...
journal_title：Clinical biochemistry
pub_type： 杂志文章,多中心研究
doi：10.1016/0009-9120(93)90017-z
更新日期：1993-02-01 00:00:00
abstract：:An affinity chromatographic method (AC) was compared with ion exchange chromatography (IEC) for determination of glycated hemoglobin (gHb). In vitro, increasing glucose concentration produced no change on AC-determined gHb. gHb measurement by AC was highly correlated with mean blood glucose (r = 0.96). Mean values of ...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/s0009-9120(86)80010-8
更新日期：1986-12-01 00:00:00
abstract：OBJECTIVES:Multiplexing technologies based on the use of microspheres as the solid phase have opened new possibilities for the analysis of autoantibodies. As an alternative to the traditional immunoassays, it is possible to use these methods in combination with flow cytometry for simultaneous measurement of anti-thyroi...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2005.08.004
更新日期：2005-11-01 00:00:00
abstract：:Mycophenolate mofetil is widely used in combination with either cyclosporine or tacrolimus for rejection prophylaxis in renal and heart transplant patients. Although not monitored routinely nearly to the degree that other agents such as cyclosporine or tacrolimus, there is an expanding body of experimental evidence fo...
journal_title：Clinical biochemistry
pub_type： 杂志文章,评审
doi：10.1016/s0009-9120(00)00184-3
更新日期：2001-02-01 00:00:00
abstract：OBJECTIVES:Abnormal alanine aminotransferase (ALT) activity is indicative of liver disease even a burden of overall health. We assessed the factors associated with ALT activity and their internal relationships in a male population from southern China. DESIGN AND METHODS:Data of physical examinations, laboratory tests,...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2012.08.022
更新日期：2012-12-01 00:00:00
abstract：INTRODUCTION:In the Olympus uric acid procedure, uric acid is converted by uricase to allantoin and hydrogen peroxide, which is reacted in a Trinder reaction to produce a chromophore read bichromatically at 520 and 660 nm. Repeated difficulty was encountered in obtaining uric acid results on samples from myeloma patien...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/s0009-9120(98)00064-2
更新日期：1998-10-01 00:00:00
abstract：OBJECTIVES:There has been growing interest in the use of serum B-type natriuretic peptide (BNP) and the N-terminal segment of its pro-hormone (NT-proBNP) as biomarkers for cardiac disease. The aim of this review is to summarize the current state of knowledge regarding BNP and NT-proBNP measurement in the pediatric popu...
journal_title：Clinical biochemistry
pub_type： 杂志文章,评审
doi：10.1016/j.clinbiochem.2005.12.004
更新日期：2006-06-01 00:00:00
abstract：:This article reviews some of the important determinants of variation in drug disposition such as age, gender, body weight, diet, environmental influences, drug - protein interactions, compliance, drug - drug interactions, endogenous substances, disease states, circadian variation and genetics. ...
journal_title：Clinical biochemistry
pub_type： 杂志文章,评审
doi：10.1016/s0009-9120(86)80050-9
更新日期：1986-04-01 00:00:00
abstract：OBJECTIVES:Breast cancer is the most common malignancy among Egyptian women. The aim of this study is to evaluate the role of both telomerase and TPS estimation in assessment of breast cancer. METHODS:The study included 40 patients with breast cancer, and 20 patients with benign breast diseases. Telomerase activity in...
journal_title：Clinical biochemistry
pub_type： 临床试验,杂志文章
doi：10.1016/s0009-9120(01)00253-3
更新日期：2001-09-01 00:00:00
abstract：:We present a systematic parametric method for resolving age-related reference intervals based on differential-polynomial regression analysis. Application of this method to plasma zinc concentrations in a childhood population results in a third degree polynomial expression from which two statistically independent age-r...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/s0009-9120(79)90056-0
更新日期：1979-02-01 00:00:00
abstract：:A non-isotopic heterogeneous non-competitive immunoassay for carcinoembryonic antigen is described. CEA present in serum binds simultaneously to two monoclonal antibodies specific for different antigenic determinants of CEA. One antibody is immobilized on a solid-phase (microtiter well) and the other is biotinylated. ...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/s0009-9120(89)80094-3
更新日期：1989-12-01 00:00:00
abstract：:Fluoride is a potent therapeutic agent that increases spinal bone density in osteoporotic subjects. Based on work with animal cells previously, we proposed fluoride acts by inhibiting phosphotyrosyl protein phosphatase (EC 3.1.3.48) activity in bone cells. The presence of fluoride sensitive acid phosphatase (EC 3.1.3....
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/0009-9120(92)80045-i
更新日期：1992-02-01 00:00:00
abstract：OBJECTIVES:To test for specific mutations in the alanine:glyoxylate aminotransferase (AGT) gene, in order to diagnose primary hyperoxaluria type 1 (PH1). DESIGN AND METHODS:Samples of liver and/or DNA from 81 patients were submitted to our laboratory for diagnostic testing for PH1. Using a panel of selected mutations,...
journal_title：Clinical biochemistry
pub_type： 杂志文章,多中心研究
doi：10.1016/j.clinbiochem.2008.01.018
更新日期：2008-05-01 00:00:00
abstract：OBJECTIVES:Osteoprotegerin (OPG) is a regulator of bone and vascular homeostasis and acts as a decoy receptor for proapoptotic TNF-related apoptosis-inducing ligand (TRAIL). DESIGN AND METHODS:We assessed pericardial and serum levels of OPG and TRAIL in pericardial effusions (PE) of malignant (mPE, n=24) or non-malign...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2011.12.003
更新日期：2012-02-01 00:00:00
abstract：OBJECTIVES:This study aimed to examine the association between promoter polymorphisms of Th1 and Th2 cytokine genes [interleukin-4 (IL-4 T-34C, A-81G, C-285T and T-589C), IL-6 (G-174C), IL-10 (A-592C and T-819C) and tumour necrosis factor-alpha (TNF-alpha G-238A and G-308A)] and Graves' disease (GD) in Taiwanese popula...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2006.11.009
更新日期：2007-02-01 00:00:00
abstract：OBJECTIVES:To test the hypothesis that impaired expression of the thiosulfate sulfurtransferase rhodanese is associated with oxidative stress and may predict mortality in hemodialysis patients. DESIGN AND METHODS:Sixty-two hemodialysis patients were investigated to determine protein and mRNA expression of rhodanese in...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2009.08.005
更新日期：2010-01-01 00:00:00
abstract：OBJECTIVES:To produce a monoclonal antibody (MAb) against electronegative LDL (LDL-) for detecting this modified lipoprotein in blood plasma and tissues. DESIGN AND METHODS:LDL- was isolated from human blood plasma and used as an antigen for immunization of Balb/c mice. Lymphocytes of immunized mice were fused with my...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2005.09.014
更新日期：2006-01-01 00:00:00
abstract：:Steroids play an important role in sperm production and quality. These hormones have been extensively studied in blood, but poorly investigated in semen. The purpose of our study was to evaluate the relationship between sperm quality and steroid profiles in blood and semen in a small cohort of young Swiss men. Another...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2018.03.008
更新日期：2018-12-01 00:00:00
