Abstract：
OBJECTIVE:Saposin C (SAP-C) is an essential activator of glucosylceramide (GlcCer)-β-glucosidase (GCase), the enzyme deficient in Gaucher's disease. In this study, we investigated the effects of chemically synthesized SAP-Cs (synthetic SAP-Cs) on GCase. METHODS:Enzymatic assays and western blot analyses were carried out to evaluate the effects of two kinds of synthetic SAP-Cs, a non-glycosylated form and a N-glycosylated form bearing a complex type nonasaccharide, on GCase with respect to its activation, stabilization, and protection. Imiglucerase (Cerezyme) was used as the GCase. To mimic physiological conditions, GCase activity was assayed in the presence of 4-nitrobenzo-2-oxa-1,3-diazole-labeled GlcCer-containing liposomes composed of bis(monoacylglycero)phosphate, l-α-phosphatidylcholine, and cholesterol. RESULTS:GCase activities increased depending on the concentration of synthetic SAP-Cs. SAP-Cs at a concentration of 1μM increased GCase activities significantly, by 14- to 22-fold (non-glycosylated SAP-C: 22.9±0.16; nona-glycosylated SAP-C: 14.9±0.19; without SAP-C: 1.05±0.035pmol/h/ng GCase). These values equaled or surpassed previously published values obtained using recombinant non-glycosylated SAP-C. Both synthetic SAP-Cs were as effective as bovine serum albumin in stabilizing GCase at 37°C. Western blot analysis revealed that synthetic SAP-Cs specifically protected GCase from cathepsin D digestion. CONCLUSIONS:The results demonstrate that these novel, chemically synthesized SAP-Cs function as activators, stabilizers, and protectors of GCase, suggesting their utility in enzyme replacement therapy in patients with Gaucher's disease.
journal_name
Clin Biochemjournal_title
Clinical biochemistryauthors
Yoneshige A,Muto M,Watanabe T,Hojo H,Matsuda Jdoi
10.1016/j.clinbiochem.2015.06.004subject
Has Abstractpub_date
2015-11-01 00:00:00pages
1177-80issue
16-17eissn
0009-9120issn
1873-2933pii
S0009-9120(15)00217-9journal_volume
48pub_type
杂志文章abstract：OBJECTIVES:Carbohydrate-deficient transferrin CDT has originally been defined as the sum of isotransferrins exhibiting isoelectric point values > or = 5.7 asialo, monosialo, and disialo transferrin but may also include at least in part trisialo transferrin when measured by modern commercial immunoassays. To examine the...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/s0009-9120(00)00189-2
更新日期：2000-11-01 00:00:00
abstract：OBJECTIVE:Mycophenolic acid requires routine therapeutic drug monitoring. We evaluated the suitability of a new PETINIA (particle enhanced turbidimetric inhibition immunoassay) assay on the Dimension EXL analyzer for monitoring of mycophenolic acid by comparing values obtained by this assay with a HPLC-UV method. DESI...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2012.11.025
更新日期：2013-05-01 00:00:00
abstract：:Epidemiological evidence suggests that the ratio HDL-cholesterol (HDL-C)/total cholesterol (TC) or apolipoprotein A1 (apo A1)/apolipoprotein B (apo B) are good indicators of coronary heart disease risk. In investigating the distribution of these ratios in the typical population served by our routine laboratory, we ana...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/0009-9120(92)80004-z
更新日期：1992-10-01 00:00:00
abstract：OBJECTIVES:BNP and NT-proBNP are viewed as comparable in their ability to diagnose and monitor HF in clinical guidelines. However, no recent large-scale study has directly established diagnostic concordance between BNP and NT-proBNP. This study sought to assess diagnostic concordance of BNP and NT-proBNP for ruling in ...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2018.07.002
更新日期：2018-09-01 00:00:00
abstract：OBJECTIVES:We investigated the stability of 36 analytes related to clinical chemistry in a controlled storage study. DESIGN AND METHODS:Blood was collected from 11 subjects and was maintained for 45 min, 2.5 h, 5 h, or 24 h after phlebotomy before centrifugation. RESULTS:Statistically significant changes were observe...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2009.02.010
更新日期：2009-06-01 00:00:00
abstract：OBJECTIVES:We previously described a panel of four cytokines biomarkers in knee synovial fluid for acute knee pain associated with meniscal pathology. The cytokine biomarkers included interferon gamma (IFN-gamma), interleukin 6 (IL-6), monocyte chemotactic protein 1 (MCP-1), and macrophage inflammatory protein-1 beta (...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2010.04.069
更新日期：2010-07-01 00:00:00
abstract：BACKGROUND:Measurement of 25(OH)D has evolved rapidly, with an increased number of high-throughput automated immunoassays becoming available in recent years. The aim of this study was to fully evaluate six commercially available automated 25(OH)D immunoassays, including 2 newly available (Beckman Coulter) and one recal...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2015.08.005
更新日期：2015-11-01 00:00:00
abstract：OBJECTIVE:Evaluation of serum beta-2-microglobulin (beta2M) in an automated analyzer. DESIGN AND METHODS:The DakoCytomation beta2M kit is an antibody based reagent intended for quantitative determination of beta2M in serum and plasma by rate nephelometry. RESULTS:The limit of blank is 0.16 mg/L. The method is linear ...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2009.06.030
更新日期：2009-10-01 00:00:00
abstract：OBJECTIVES:Elevated advanced glycation endproducts (AGEs) are implicated in diabetic complications. Methylglyoxal-derived hydroimidazolone (MG-H) is one of the most abundant AGEs in vivo. Our objective was to develop a time-saving, specific method to measure free MG-H in plasma and determine its levels in complication-...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2008.12.016
更新日期：2009-05-01 00:00:00
abstract：OBJECTIVES:We established population-based reference limits for plasma total homocysteine (tHcy) according to creatinine. DESIGN AND METHODS:In 7042 middle-aged and elderly subjects from the Hordaland Homocysteine Study, we used statistical modeling to establish nomograms for tHcy according to creatinine in the whole ...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2007.07.014
更新日期：2007-11-01 00:00:00
abstract：OBJECTIVES:The diagnosis of pheochromocytoma relies on the measurement of plasma free metanephrines assay whose reliability has been considerably improved by ultra-high pressure liquid chromatography tandem mass spectrometry (UHPLC-MS/MS). Here we report an analytical interference occurring between 4-hydroxy-3-methoxym...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2014.04.003
更新日期：2014-08-01 00:00:00
abstract：OBJECTIVES:The ability of current immunoassays to accurately measure equimolar amounts of 25(OH)D2 and 25(OH)D3 has been recently questioned. This study determined serum 25(OH)D2, 25(OH)D3 and total serum 25(OH)D concentrations in healthy vitamin D2-supplemented subjects by isotope dilution liquid chromatography mass s...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2015.05.021
更新日期：2015-11-01 00:00:00
abstract：OBJECTIVE:Plasma ammonia measurement is greatly influenced by pre-analytical conditions, which may lead to false positives. We wanted to evaluate the prevalence and clinical impact of plasma ammonia false positives in a pediatric population. DESIGN AND METHODS:Over a 28-month period, charts of patients with elevated a...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2007.01.024
更新日期：2007-05-01 00:00:00
abstract：OBJECTIVES:Reporting a case of inherited factor V deficiency and galactosemia. METHODS:A neonate was admitted with hematoma, jaundice, splenomegaly, diarrhea, anemia, abdominal ascites and bilateral cataracts that diagnosis of galactosaemia and factor V deficiency was established. RESULTS:Coinheritance of both coagul...
journal_title：Clinical biochemistry
pub_type： 杂志文章,评审
doi：10.1016/j.clinbiochem.2011.12.024
更新日期：2012-03-01 00:00:00
abstract：OBJECTIVES:The present study was designed to (a) evaluate and compare plasma ammonia levels (PAL) in patients with acute liver failure (ALF) and chronic liver disease (CLD) with or without hepatic encephalopathy (HE); (b) correlate the severity of HE with PAL; and (c) correlate PAL with clinical features of raised intr...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2005.04.013
更新日期：2005-08-01 00:00:00
abstract：:Increased hemoglobin A1 (HbA1) occurs in diabetes mellitus as a direct consequence of hyperglycemia, which causes postsynthetic modification of hemoglobin proportional to blood glucose concentration. Glycosylation of hemoglobin is demonstrable also in vitro, not only by glucose, but with other reducing sugars such as ...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/s0009-9120(82)90556-2
更新日期：1982-06-01 00:00:00
abstract：BACKGROUND:Currently, 20 IU/L is the accepted sensitivity of over-the-counter (OTC) hCG pregnancy tests, and the only sensitivity permitted for point of care (POC) tests. These sensitivity limitations cause significant numbers of false-negative tests, which mislead women and cause them to avoid precautions needed to av...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2008.09.107
更新日期：2009-02-01 00:00:00
abstract：OBJECTIVES:To ascertain whether or not the change in blood collection tubes for plasma glucose from fluoride/oxalate to citrate/fluoride/EDTA has had an effect upon the glucose results. METHODS:Plasma glucose results from fasting patients from 2007 to 2012 were extracted from the laboratory information system. The dat...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2014.01.011
更新日期：2014-05-01 00:00:00
abstract：:The initial step in the action of thyrotropin (TSH) is its binding to the TSH receptor. TSH receptor antibodies are detected in up to 90% of patients with Graves' disease. Serial measurements of TSH receptor antibodies in patients with Graves' hyperthyroidism are helpful in predicting relapse. The TSH receptor was pur...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/0009-9120(90)90417-s
更新日期：1990-02-01 00:00:00
abstract：:To elucidate the diagnostic relevance of biliary conjugated bilirubin, biliary bilirubin from normal volunteers (NV), patients with Gilbert's syndrome (GS) and Crigler-Najjar syndrome type II (C-N II), and from various rat strains was fractionated. Biliary bilirubin diglucuronide (BDG) was present at lower levels, and...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/0009-9120(90)80024-d
更新日期：1990-04-01 00:00:00
abstract：OBJECTIVES:The plasma apolipoprotein B (apo B) concentrations have been considered to be a more accurate representation of atherogenic particles and it has been proposed that the formula LDL-C (mmol/L) = 0.41TC - 0.32TG + 1.70apo B - 0.27 is reliable for the estimation of LDL-C (Clin Chem 1997; 43: 808-15). We undertoo...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/s0009-9120(00)00162-4
更新日期：2000-10-01 00:00:00
abstract：OBJECTIVES:Personalized ranges of liver fibrosis serum biomarkers such as FibroTest or hyaluronic acid could be used for early detection of fibrotic changes in patients with progressive chronic liver disease. Our aim was to generate reliable biological variation estimates for these two biomarkers with confidence interv...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2015.03.020
更新日期：2015-07-01 00:00:00
abstract：OBJECTIVES:The aim of this study was to determine whether the atherogenic index of plasma (AIP=log[triglycerides/HDL-cholesterol]) differs in heterozygous familial hypercholesterolemia (FH) patients with and without a history of cardiovascular disease (CVD). DESIGN AND METHODS:A total of 555 FH patients with known mut...
journal_title：Clinical biochemistry
pub_type： 杂志文章,多中心研究
doi：10.1016/j.clinbiochem.2011.11.001
更新日期：2012-01-01 00:00:00
abstract：OBJECTIVES:Classical galactosemia (McKusick 230400) is an autosomal recessive disorder caused by mutations in the galactose-1-phosphate uridyl transferase (GALT;EC 2.7.7.10) gene. DESIGN AND METHODS:In the present study, we report molecular analysis of 14 unrelated Iranian galactosemia children with reduced or without...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2006.04.020
更新日期：2006-07-01 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：UNLABELLED:Antioxidant capacity testing is commonly used in clinical investigations to provide an estimate of in vitro antioxidant capacity of biosamples. Although beneficial to measure the synergistic contribution of all compounds with antioxidant functionality, assessing the capacity of non-protein fractions or small...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/j.clinbiochem.2014.06.021
更新日期：2014-10-01 00:00:00
abstract：OBJECTIVE:We used single-strand conformational polymorphism (SSCP). To screen for mutations/polymorphisms in exon 4 of the apolipoprotein C III in 45 patients with hypertriglyceridemia and 46 control individuals, single-strand conformational polymorphism was investigated using restriction endonuclease and amplification...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/0009-9120(94)00083-8
更新日期：1995-06-01 00:00:00
abstract：:We evaluated two chemical methods for quantifying mannitol in serum, based on the oxidation of mannitol by periodate, and measurement of the formaldehyde formed with chromotropic acid (colorimetry) or acetylacetone (fluorometry). We found interference in these methods by serum glycerol. Additionally, a high-performanc...
journal_title：Clinical biochemistry
pub_type： 杂志文章
doi：10.1016/0009-9120(92)90120-h
更新日期：1992-12-01 00:00:00
abstract：:Several clinical entities are associated with disorders of fatty acid oxidation or transfer across the inner mitochondrial membrane. Over 40 cases of the primary carnitine deficiency syndrome have been reported to date and various subtypes have been characterized. This represents a large clinical spectrum. The deficie...
journal_title：Clinical biochemistry
pub_type： 杂志文章,评审
doi：10.1016/s0009-9120(87)80090-5
更新日期：1987-02-01 00:00:00
abstract：OBJECTIVES:We evaluated the effects of lifestyle modification (LM) on lipid profile, oxidative stress and serum-stimulated human coronary artery endothelial cell (HCAEC) viability in coronary artery disease (CAD) patients after 6months. DESIGN AND METHODS:Thirty patients with CAD were randomly assigned to LM intervent...
journal_title：Clinical biochemistry
pub_type： 杂志文章,随机对照试验
doi：10.1016/j.clinbiochem.2010.04.056
更新日期：2010-07-01 00:00:00
