An in situ study of beta-glucosidase activity in normal and Gaucher fibroblasts with fluorogenic probes.

Abstract:

:Beta-glucosidase activity was evaluated in situ by means of fluorogenic probes in normal human fibroblasts and fibroblasts from homozygous carriers of the Gaucher trait. Probe internalization, targeting to lysosomes and post-cleavage probe retention were the primary concerns. Internalization and targeting were attempted by in situ photosensitized labilization of lysosomal membranes, lysosomotropic detergents and the use of low density lipid (LDL) or the receptor ligand apolipoprotein E (ApoE). Post-cleavage increase of fluorescence with fluoresceinyl (bis) beta-glucopyranoside was appreciably above the rather large pre-cleavage emission. In cells incubated overnight with nonylumbelliferyl-beta-glucoside (UG9) in the presence of bovine serum albumin and in the absence of ApoE, the probe was dealt with as a cytotoxic agent, accumulating in a paranuclear cap, most likely comprising elements of the endoplasmic reticulum (ER) and Golgi apparatus. Targeting of UG9 to lysosomes occurred within 1 to 3 h of preincubation in the presence of ApoE. There was some evidence of specificity, as Gaucher fibroblasts exhibited weaker cleavage of UG9 (by 50 per cent or more) compared to normal fibroblasts, but in the Gaucher cells there was some residual beta-glucosidase activity. Cleavage of UG9 was nearly totally suppressed in Gaucher cells treated with the beta-glucosidase inhibitor, conduritol B epoxide, for 24 h to 7 days. Suppression in the control fibroblasts was evident but to a lesser degree. The in situ method of fluorogenic assay established for beta-glucosidase deficiency, is in principle applicable to enzyme deficiencies in other lysosomal storage diseases, or to evaluate enhanced enzyme activity following gene therapy.

journal_name

Cell Biochem Funct

authors

Kohen E,Kohen C,Hirschberg JG,Santus R,Grabowski G,Mangel W,Gatt S,Prince J

doi

10.1002/cbf.290110304

subject

Has Abstract

pub_date

1993-09-01 00:00:00

pages

167-77

issue

3

eissn

0263-6484

issn

1099-0844

journal_volume

11

pub_type

杂志文章
  • Alteration of phosphotyrosine-containing proteins during differentiation of chicken erythroleukemia cells (HD3).

    abstract::After treatment of HD3 cells with erythroid-inducing agents (hemin and butyric acid) at 42 degrees C, the profile of phosphotyrosine-containing proteins was altered. Upon induction the overall level of phosphotyrosine-containing proteins increased. To examine the role of protein phosphorylation in HD3 cells differenti...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/(SICI)1099-0844(1998120)16:4<277::AID-CBF7

    authors: Grdisa M

    更新日期:1998-12-01 00:00:00

  • Potency and property of DNA conformational damage determine ATM activation: two new DNA topological probes.

    abstract::We have shown previously the intercalation geometry of a series of acenaphtho [1,2-b] pyrrole derivatives with DNA double helix in vitro. In this report we chose a couple of intercalating analogues and a Chinese traditional medicine Tanshinone IIA as probes to investigate the response of DNA damage sensor ataxia-telan...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.1510

    authors: Zhang Z,Wang Y,Zhang J,Song T,Qian X

    更新日期:2008-10-01 00:00:00

  • Lipid peroxidation and antioxidant enzyme activities in erythrocytes of type I and II alcoholics.

    abstract::Reduced and oxidized glutathione (GSH and GSSG), protein-bound glutathione, lipid peroxidation and antioxidant enzyme activities were determined in the erythrocyte lysates and membranes of type I and II alcoholics in order to clarify the effect of age-of-onset and the duration of the alcohol consumption on erythrocyte...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.1125

    authors: Uçar G,Demir B,Uluğ B

    更新日期:2005-01-01 00:00:00

  • Role of Ca2+/calmodulin-dependent protein kinase II in development of vascular dysfunction in diabetic rats with hypertension.

    abstract::We examined the influence of chronic treatment with KN-93 (an inhibitor of Ca(2+)/calmodulin-dependent protein kinase II (CaMKII), 5 mg/kg given every other day for 4 weeks) on mean arterial pressure (MAP), urine protein and vascular reactivity in streptozotocin (STZ)-induced diabetes in Wistar Kyoto (WKY) and spontan...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.1446

    authors: Yousif MH,Akhtar S,Walther T,Benter IF

    更新日期:2008-03-01 00:00:00

  • Antimicrobial and biological effects of ipemphos and amphos on bacterial and yeast strains.

    abstract::In this study, the antimicrobial effects of monophosphazenes such as SM ipemphos and amphos were examined on bacterial and yeast strains. In addition, the biological effects of these compounds were tested on the lipid level of Saccharomyces cerevisiae and Candida albicans cells. The SM has an antimicrobial effect on t...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/(SICI)1099-0844(200006)18:2<117::AID-CBF86

    authors: Oztürk AI,Yilmaz O,Kirbağ S,Arslan M

    更新日期:2000-06-01 00:00:00

  • Proliferation- and migration-enhancing effects of ginseng and ginsenoside Rg1 through IGF-I- and FGF-2-signaling pathways on RSC96 Schwann cells.

    abstract::The aim of the present study is to evaluate the proliferation- and migration-enhancing effects of ginseng and its component, ginsenoside (Rg1) on RSC96 Schwann cells. We investigated the molecular signaling pathways, which include: (1) survival signaling, IGFs-IGFIR-Akt-Bcl2 and proliferative signaling, cell cycle fac...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.1554

    authors: Lu MC,Lai TY,Hwang JM,Chen HT,Chang SH,Tsai FJ,Wang HL,Lin CC,Kuo WW,Huang CY

    更新日期:2009-06-01 00:00:00

  • Role of protein kinases in mediating diabetes-induced augmented vasoconstriction to endothelin-1 in the renal arteries of STZ-diabetic rats.

    abstract::Diabetes is associated with increased reactivity of the renal vascular bed to endothelin-1 (ET-1). It has been observed that diabetes is associated with over-expression of ET(A)- and ET(B)-receptors in the rat renal cortex. However it is not known if these receptors are over-expressed in the renal artery. The objectiv...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.1253

    authors: Yousif MH

    更新日期:2006-09-01 00:00:00

  • Cervico-vaginal dysplasia--papillomavirus-induced and HIV-1 infection: role of correlated markers for prognostic evaluation.

    abstract::Sexually-transmitted diseases (STD) can facilitate the progression of HIV-1 infection. Among them, as we have previously demonstrated, cervico-vaginal dysplasia-papillomavirus (HPV)-induced, together with HSV-2 co-infection, seems to be correlated with a more evident immunodepression in HIV-positive women, compared wi...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.950

    authors: Pugliese A,Andronico L,Gennero L,Pagliano G,Gallo G,Torre D

    更新日期:2002-09-01 00:00:00

  • Cloturin: effect on energy-producing processes in Ehrlich ascites and P388 murine leukaemia cells.

    abstract::The main purpose of the present investigation was to study the effect of cloturin on aerobic glycolysis, endogenous and exogenous respiration and the level of ATP in both Ehrlich ascites carcinoma (EAC) and P388 murine leukaemia cells incubated in vitro. Also its effect on the level of total (T-SH) and non-protein (NP...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.290100208

    authors: Miko M,Krepelka J,Melka M

    更新日期:1992-06-01 00:00:00

  • The effect of taurine supplementation on oxidative stress in experimental hypothyroidism.

    abstract::The purpose of this study was to investigate the oxidative status in experimental hypothyroidism and the antioxidant effect of taurine supplementation. Forty male Sprague Dawley rats were randomly divided into four groups (group 1, control; group 2, control + taurine; group 3, propylthiouracil (PTU); group 4, PTU + ta...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.1198

    authors: Taş S,Dirican M,Sarandöl E,Serdar Z

    更新日期:2006-03-01 00:00:00

  • Effects of thymoquinone on antioxidant enzyme activities, lipid peroxidation and DT-diaphorase in different tissues of mice: a possible mechanism of action.

    abstract::The present investigation focused, firstly, on the effects of oral administration of thymoquinone (TQ) on antioxidant enzyme activities, lipid peroxidation and DT-diaphorase activity in hepatic, cardiac and kidney tissues of normal mice. Superoxide dismutase (SOD; E.C:1.15.1.1), catalase (CAT; E.C:1.11.1.6), glutathio...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.968

    authors: Mansour MA,Nagi MN,El-Khatib AS,Al-Bekairi AM

    更新日期:2002-06-01 00:00:00

  • Triiodothyronine receptor sites in serum-free cultured hepatocytes from adult rat liver.

    abstract::Nuclear T3 specific binding sites were characterized by Scatchard analyses of L-125I-T3 binding to nuclei extracted from freshly isolated and 1, 2 and 6 day-cultured hepatocytes. The results demonstrate a marked decrease in T3 binding capacity of nuclei extracted from 1 day-cultured cells followed by an almost complet...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.290030203

    authors: Goglia F,Gallo G,Palmero S,Voci A,Fugassa E

    更新日期:1985-04-01 00:00:00

  • Fibronectin is not a serum spreading factor for HeLa cells.

    abstract::The spreading of HeLa cells on plastic substratum is mediated by fibronectin-depleted foetal calf serum but not by fibronectin isolated by gelatin-Sepharose affinity chromatography. The same is true for freshly explanted chick embryonic chondrocytes. In contrast, BHK cell spreading exceeds 67% after 120 min at 37 degr...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.290010208

    authors: Michl J,Baudysová M,Sochorová L,Deyl Z

    更新日期:1983-07-01 00:00:00

  • Tissue malondialdehyde and adenosine triphosphatase level after experimental liver ischaemia-reperfusion damage.

    abstract::Functional irregularities due to damage after ischaemia-reperfusion vary depending upon the organs affected. High energy phosphates such as ATP and ADP are destroyed after ischaemia-reperfusion damage. Subsequently, protons and inorganic phosphates accumulate within the cells and the proton pumps such as adenosine tri...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.912

    authors: Ilhan N,Halifeoglu I,Ozercan HI,Ilhan N

    更新日期:2001-09-01 00:00:00

  • Effects of melatonin on oxidative-antioxidative status of tissues in streptozotocin-induced diabetic rats.

    abstract::In view of the antioxidant properties of melatonin, the effects of melatonin on the oxidative-antioxidative status of tissues affected by diabetes, e.g. liver, heart and kidneys, were investigated in streptozotocin (STZ)-induced diabetic rats in the present study. Concentrations of malondialdehyde (MDA) and reduced gl...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.1006

    authors: Aksoy N,Vural H,Sabuncu T,Aksoy S

    更新日期:2003-06-01 00:00:00

  • Age-dependent changes in in vivo ethanol metabolism and in the activity of hepatic enzymes involved in ethanol oxidation and microsomal functions.

    abstract::The study of the influence of the age of the animals (13 to 53 weeks) on the rate of ethanol metabolism in vivo and the total activity of liver alcohol dehydrogenase and microsomal ethanol oxidizing system showed a progressive decline with age. These effects were observed concomitantly with a diminution in the content...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.290060103

    authors: Fernandez V,Kriz A,Videla LA

    更新日期:1988-01-01 00:00:00

  • Functional analysis of mesencephalic astrocyte-derived neurotrophic factor in retinal ganglion cells under oxidative stress.

    abstract::Glaucoma is optic neuropathy that is characterized by progressive neurodegeneration of the retinal ganglion cells (RGCs) and axons. This condition will lead to visual impairment and bring glaucoma to become the second cause of blindness globally. Neuroprotection in glaucoma is needed to prevent the progression of opti...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.3567

    authors: Ko JA,Komatsu K,Okumichi H,Kiuchi Y

    更新日期:2021-01-01 00:00:00

  • Isolation and characterization of mesenchymal stem cells from whole human umbilical cord applying a single enzyme approach.

    abstract::Mesenchymal stem cells (MSCs) are multipotent cells traditionally derived from bone marrow (BM). They have been demonstrated to be widely applied in tissue regeneration and cellular therapy. As an alternative to BM, an umbilical cord (UC) is considered as a potential source of MSCs. Here, we showed that human UC-MSCs ...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.2843

    authors: Zhang H,Zhang B,Tao Y,Cheng M,Hu J,Xu M,Chen H

    更新日期:2012-12-01 00:00:00

  • Oxygen free radical scavenger properties of dehydroepiandrosterone.

    abstract::The microsomes from dehydroepiandrosterone (DHEA)-supplemented animals are good hydroxyl radical scavengers, as demonstrated through electron spin resonance and deoxyribose degradation. The ability of DHEA-supplemented microsomes to react with superoxide radical was also demonstrated through the inhibition of nitroblu...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/(SICI)1099-0844(199803)16:1<57::AID-CBF771

    authors: Tamagno E,Aragno M,Boccuzzi G,Gallo M,Parola S,Fubini B,Poli G,Danni O

    更新日期:1998-03-01 00:00:00

  • Adenine nucleotides in thrombocytes of birds.

    abstract::Analysis of free nucleotide composition of both avian thrombocytes and pig platelets showed quantitative differences in the level of adenine nucleotides. 3H-adenine taken up by turkey thrombocytes was metabolized mainly to adenine nucleotides was not released after thrombin action. Thrombin liberated non-radioactive a...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.290020310

    authors: Wachowicz B

    更新日期:1984-07-01 00:00:00

  • Genetic instability of RBM5/LUCA-15/H37 in MCF-7 breast carcinoma sublines may affect susceptibility to apoptosis.

    abstract::The MCF-7 human breast carcinoma cell line is widely used as a model system by breast cancer researchers and cell biologists investigating apoptosis. Since its establishment 30 years ago, from a patient with metastatic breast cancer, the original MCF-7 cell population has undergone genetic drift to such an extent that...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.1106

    authors: Rintala-Maki ND,Abrasonis V,Burd M,Sutherland LC

    更新日期:2004-09-01 00:00:00

  • Stimulation of human platelets with concanavalin A involves phospholipase C activation.

    abstract::In response to concanavalin A, cytoplasmic calcium movement was observed in human platelets, both in the presence of 1 mM Ca2+ or 1 mM EGTA in the medium. Concanavalin A also caused the activation of inositide turnover and the production of inositol phosphates, suggesting that activation of phospholipase C occurs. The...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.290100109

    authors: Torti M,Balduini C,Ramaschi G,Sinigaglia F

    更新日期:1992-03-01 00:00:00

  • Anti-ageing effects of protocatechuic acid from Alpinia on spleen and liver antioxidative system of senescent mice.

    abstract::The effects of Alpinia protocatechuic acid (PCA) on spleen and liver antioxidant system in aged rats have been studied. Alpinia PCA, a phenolic compound, was first isolated from the dried fruits of Alpinia Oxyphylla Miq. in our laboratory. Young and aged rats were injected intraperitoneally with Alpinia PCA at single ...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.1757

    authors: Zhang X,Shi GF,Liu XZ,An LJ,Guan S

    更新日期:2011-06-01 00:00:00

  • Cytochrome P450 in highly purified suspension of nonparenchymal liver cells.

    abstract::Rat liver nonparenchymal cells (NPC) were prepared by pronase digestion and purified on discontinuous gradients on Nycodenz. Morphological and biochemical characterization of cell suspensions showed that they were free of contamination by hepatocytes. We have confirmed the usefulness of pyruvate kinase activity in mon...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.290070406

    authors: Rich K,Lodola A

    更新日期:1989-10-01 00:00:00

  • The importance of hormone receptor analysis in osteosarcoma cells growth submitted to treatment with estrogen in association with thyroid hormone.

    abstract::Bone tumor incidence in women peaks at age 50-60, coinciding with the menopause. That estrogen (E2) and triiodothyronine (T3) interact in bone metabolism has been well established. However, few data on the action of these hormones are available. Our purpose was to determine the role of E2 and T3 in the expression of b...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.1408

    authors: Saraiva PP,Teixeira SS,Conde SJ,Nogueira CR

    更新日期:2008-01-01 00:00:00

  • Ethanol-induced hepatotoxicity and protective effect of betaine.

    abstract::The protective effects of betaine in ethanol hepatotoxicity were investigated in 24 female wistar albino rats. Animals were divided into three groups: control, ethanol and ethanol + betaine group. Animals were fed liquid diets and consumed approximately 60 diet per day. Rats were fed ethanol 8 kg(- 1) day(- 1). The et...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.926

    authors: Kanbak G,Inal M,Bayçu C

    更新日期:2001-12-01 00:00:00

  • Trikatu, a herbal compound that suppresses monosodium urate crystal-induced inflammation in rats, an experimental model for acute gouty arthritis.

    abstract::Gout is an inflammatory joint disorder characterized by hyperuricaemia and precipitation of monosodium urate crystals in the joints. In the present study, we aimed to investigate the anti-inflammatory effect of trikatu, a herbal compound in monosodium urate crystal-induced inflammation in rats, an experimental model f...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.2979

    authors: Murunikkara V,Rasool M

    更新日期:2014-01-01 00:00:00

  • Mitochondria as the target for mildronate's protective effects in azidothymidine (AZT)-induced toxicity of isolated rat liver mitochondria.

    abstract::Previously mildronate, an aza-butyrobetaine derivative, was shown to be a cytoprotective drug, through its mechanism of action of inhibition of carnitine palmitoyltransferase-1, thus protecting mitochondria from long-chain fatty acid accumulation and subsequent damage. Recently in an azidothymidine (AZT)-induced cardi...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.1486

    authors: Pupure J,Fernandes MA,Santos MS,Moreno AJ,Kalvinsh I,Klusa V,Oliveira CR

    更新日期:2008-09-01 00:00:00

  • Chemoprotective effects of carnosine against genotoxicity induced by cyclophosphamide in mice bone marrow cells.

    abstract::The protective effects of carnosine as a natural dipeptide were investigated in mouse bone marrow cells against genotoxicity induced by cyclophosphamide. Mice were injected with solutions of carnosine at three different doses (10, 50 and 100 mg kg(-1) bw) for five consecutive days. On the fifth day of treatment, mice ...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章

    doi:10.1002/cbf.2834

    authors: Naghshvar F,Abianeh SM,Ahmadashrafi S,Hosseinimehr SJ

    更新日期:2012-10-01 00:00:00

  • Role of fatty acids in the transition from anaerobic to aerobic metabolism in skeletal muscle during exercise.

    abstract::In moderate physical exercise, the transition from predominantly anaerobic towards predominantly aerobic metabolism is a key step to improve performance. Increase in the supply of oxygen and nutrients, such as free fatty acids (FFA) and glucose, which accompanies high blood flow, is required for this transition. The m...

    journal_title:Cell biochemistry and function

    pub_type: 杂志文章,评审

    doi:10.1002/cbf.1327

    authors: Hirabara SM,Silveira LR,Abdulkader FR,Alberici LC,Procopio J,Carvalho CR,Pithon-Curi TC,Curi R

    更新日期:2006-11-01 00:00:00