Gene-dose effect on carnitine transport activity in embryonic fibroblasts of JVS mice as a model of human carnitine transporter deficiency.

Abstract:

:Recently, the marked decline in renal carnitine reabsorption has been thought to account fotr the systemic carnitine deficiency in juvenile visceral steatosis (JVS) mice. We have conducted a kinetic analysis using embryonic fibroblasts derived from normal, heterozygous, and homozygous jvs mice and found that the high-affinity carnitine transporter (Km = 5.5 microM), which shows Na+ and temperature dependency and stereospecificity, is defective in homozygous jvs mice. Moreover, a gene dose-dependent decrease of carnitine transport activity, which was due to a decrease in the number of the transporter molecules, was found in heterozygous jvs mice. Similar phenomena have been observed in human primary carnitine deficiency. Therefore, JVS mice may be useful for understanding this extremely rare human hereditary disorder.

journal_name

Biochem Pharmacol

journal_title

Biochemical pharmacology

authors

Hashimoto N,Suzuki F,Tamai I,Nikaido H,Kuwajima M,Hayakawa J,Tsuji A

doi

10.1016/s0006-2952(97)00670-9

subject

Has Abstract

pub_date

1998-05-15 00:00:00

pages

1729-32

issue

10

eissn

0006-2952

issn

1873-2968

pii

S0006295297006709

journal_volume

55

pub_type

杂志文章
  • The effect of chronic ritanserin and clorgyline administration on 5-HT2 receptor linked inositol phospholipid hydrolysis.

    abstract::We have previously shown that chronic administration of the 5-hydroxytryptamine (5-HT) receptor antagonist, ritanserin (10 mg/kg/day) or the monoamine oxidase type A inhibitor (MAOI), clorgyline (2 mg/kg/day), results in a reduction in 5-HT2 receptor number in rat cerebral cortex. This study investigates the effects o...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/0006-2952(90)90242-d

    authors: Twist EC,Brammer MJ,Stephenson JD,Corn TH,Campbell IC

    更新日期:1990-11-01 00:00:00

  • In vivo and in vitro peroxisome proliferation properties of selected clofibrate analogues in the rat. Structure-activity relationships.

    abstract::We have examined, relative to clofibric acid (CPIB), the effects of a chemical series of phenoxyacetic acids and of two asymmetric CPIB analogues, the R(+)- and S(-)-enantiomers of 2-(4-chlorophenoxy)propionic acid (4-CPPA) and 2-(4-chlorophenoxy)butyric acid (4-CPBA), on hepatic peroxisome proliferation both in vivo ...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/0006-2952(90)90392-x

    authors: Esbenshade TA,Kamanna VS,Newman HA,Tortorella V,Witiak DT,Feller DR

    更新日期:1990-09-15 00:00:00

  • Actin cytoskeleton, tubular sodium and the renal synthesis of dopamine.

    abstract::The present study has examined the effect of colchicine and cytochalasin B, two cytoskeleton disrupter compounds, on the formation of dopamine in slices of rat renal cortex loaded with exogenous L-3,4-dihydroxyphenylalanine (L-DOPA); the deamination of newly formed dopamine into 3,4-dihydroxyphenylacetic acid (DOPAC) ...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/0006-2952(92)90085-w

    authors: Soares-da-Silva P

    更新日期:1992-11-03 00:00:00

  • Characterization of AN6001, a positive allosteric modulator of α6β2-containing nicotinic acetylcholine receptors.

    abstract::α6β2-Containing nicotinic acetylcholine receptors (α6β2* nAChRs) are predominantly expressed in midbrain dopaminergic neurons, including substantia nigra pars compacta (SNc) neurons and their projections to striatal regions, where they regulate dopamine release and nigrostriatal activity. It is well established that n...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/j.bcp.2019.113788

    authors: van Hout M,Klein J,Ahring PK,Brown DT,Thaneshwaran S,Dos Santos AB,Jensen AA,Kohlmeier KA,Christophersen P,Dyhring T

    更新日期:2020-04-01 00:00:00

  • TGF-beta in cancer and as a therapeutic target.

    abstract::Cancer develops through a series of genetic changes leading to malignant transformation. Numerous gene and pathways involved in stages of progression to frank malignancy have been elucidated. These genetic changes result in aberrations in fundamental cellular processes controlling proliferation, apoptosis, differentia...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/j.bcp.2006.03.004

    authors: Pinkas J,Teicher BA

    更新日期:2006-08-28 00:00:00

  • PARP inhibitors: new tools to protect from inflammation.

    abstract::Poly(ADP-ribosylation) consists in the conversion of β-NAD(+) into ADP-ribose, which is then bound to acceptor proteins and further used to form polymers of variable length and structure. The correct turnover of poly(ADP-ribose) is ensured by the concerted action of poly(ADP-ribose) polymerase (PARP) and poly(ADP-ribo...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章,评审

    doi:10.1016/j.bcp.2010.04.022

    authors: Giansanti V,Donà F,Tillhon M,Scovassi AI

    更新日期:2010-12-15 00:00:00

  • Expression of acetoacetyl-CoA synthetase, a novel cytosolic ketone body-utilizing enzyme, in human brain.

    abstract::Acetoacetyl-CoA synthetase (AACS, acetoacetate-CoA ligase, EC 6.2.1.16) is a ketone body-utilizing enzyme, the physiological role of which remains unclear yet in mammals, particularly has never been studied in human. In order to investigate the tissue distribution of AACS in human, cDNA encoding AACS was isolated from...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/s0006-2952(02)01656-8

    authors: Ohgami M,Takahashi N,Yamasaki M,Fukui T

    更新日期:2003-03-15 00:00:00

  • Pyroptotic and apoptotic cell death in iNOS and nNOS overexpressing K562 cells: A mechanistic insight.

    abstract::Previous studies from this lab and others have demonstrated that nitric oxide (NO) in a concentration dependent manner, modulated neutrophil and leukemic cell survival. Subsequent studies delineated importance of iNOS in neutrophil differentiation and leukemic cell death. On the contrary, role of nNOS in survival of t...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/j.bcp.2019.113779

    authors: Sadaf S,Awasthi D,Singh AK,Nagarkoti S,Kumar S,Barthwal MK,Dikshit M

    更新日期:2020-06-01 00:00:00

  • Inhibition of nitric oxide synthase with pyrazole-1-carboxamidine and related compounds.

    abstract::Guanidines, amidines, S-alkylisothioureas, and other compounds containing the amidine function (-C(=NH)NH2) have been described as inhibitors of the generation of nitric oxide (NO) by NO synthase (NOS). Here we report on the inhibition of the activity of NOS isoforms by compounds in which the amidine function is attac...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/s0006-2952(97)00196-2

    authors: Southan GJ,Gauld D,Lubeskie A,Zingarelli B,Cuzzocrea S,Salzman AL,Szabó C,Wolff DJ

    更新日期:1997-08-01 00:00:00

  • Gene regulation of heme oxygenase-1 as a therapeutic target.

    abstract::Heme oxygenase (HO)-1 is the inducible isoform of the rate-limiting enzyme of heme degradation. HO regulates the cellular content of the pro-oxidant heme and produces catabolites with physiological functions. HO-1 is induced by a host of oxidative stress stimuli, and the activation of HO-1 gene expression is considere...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章,评审

    doi:10.1016/s0006-2952(00)00443-3

    authors: Immenschuh S,Ramadori G

    更新日期:2000-10-15 00:00:00

  • Molecular mechanisms underlying the anti-obesity potential of prunetin, an O-methylated isoflavone.

    abstract::Prunetin is an O-methylated isoflavone, which is a type of flavonoid. There are a limited number of reports detailing the biological activities of prunetin. Although an anti-inflammatory effect of prunetin has been reported in vitro, to our knowledge, there have been no reports on anti-adipogenic effects of prunetin i...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/j.bcp.2013.02.020

    authors: Ahn TG,Yang G,Lee HM,Kim MD,Choi HY,Park KS,Lee SD,Kook YB,An HJ

    更新日期:2013-05-15 00:00:00

  • Decrease in arachidonoyl-containing phosphatidylinositols in pancreas of rats fed an ethanol-containing diet.

    abstract::The composition of the glycerophosphatides in pancreas and liver was studied in rats fed an ethanol-containing diet and in pair-fed controls. The fraction of arachidonoyl-containing phosphatidylinositols in pancreas was much lower in the former rats, also when the rats were starved for a final 24 hr period. This fract...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/0006-2952(84)90521-5

    authors: Cronholm T,Curstedt T

    更新日期:1984-04-01 00:00:00

  • Contribution of phosphodiesterase isoenzymes and cyclic nucleotide efflux to the regulation of cyclic GMP levels in aortic smooth muscle cells.

    abstract::Involvement of phosphodiesterase isoenzymes (PDEs) in guanosine-3',5'-cyclic monophosphate (cGMP) hydrolysis was analyzed in aortic smooth muscle cells. Four families of PDEs were separated from pig aorta: PDE1 (calcium-calmodulin-activated), PDE3 (cGMP-inhibited), PDE4 (adenosine 3',5'-cyclic monophosphate [cAMP]-spe...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/s0006-2952(99)00252-x

    authors: Mercapide J,Santiago E,Alberdi E,Martinez-Irujo JJ

    更新日期:1999-11-15 00:00:00

  • Suppression of cytokine production and neural cell death by the anti-inflammatory alkaloid cepharanthine: a potential agent against HIV-1 encephalopathy.

    abstract::Inflammatory cytokines and human immunodeficiency virus type 1 (HIV-1) gp120 are considered to play an important role in the pathogenesis of HIV-1-associated CNS disorders. These substances are produced predominantly by HIV-1-infected or activated macrophages and microglia in the brain and induce neural cell death. Ce...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/s0006-2952(01)00692-x

    authors: Okamoto M,Ono M,Baba M

    更新日期:2001-09-15 00:00:00

  • Pentosan polysulfate to control hepcidin expression in vitro and in vivo.

    abstract::Hepcidin peptide is crucial in the regulation of systemic iron availability controlling its uptake from the diet and its release from the body storage tissues. Hepcidin dysregulation causes different human disorders ranging from iron overload (e.g. hemochromatosis) to iron deficiency (e.g. anemia). Hepcidin excess is ...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/j.bcp.2020.113867

    authors: Asperti M,Denardo A,Gryzik M,Castagna A,Girelli D,Naggi A,Arosio P,Poli M

    更新日期:2020-05-01 00:00:00

  • HIF-1: master and commander of the hypoxic world. A pharmacological approach to its regulation by siRNAs.

    abstract::The hypoxia-inducible factor-1 (HIF-1) is primarily involved in the sensing and adapting of cells to changes in the O2 level, which is essential for their viability. It is important that this critical transcription factor be tightly regulated in order for cells to respond to a wide range of O2 concentrations. HIF-1 re...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章,评审

    doi:10.1016/j.bcp.2004.04.022

    authors: Mazure NM,Brahimi-Horn MC,Berta MA,Benizri E,Bilton RL,Dayan F,Ginouvès A,Berra E,Pouysségur J

    更新日期:2004-09-15 00:00:00

  • Lawsone derivatives target the Wnt/β-catenin signaling pathway in multidrug-resistant acute lymphoblastic leukemia cells.

    abstract::Multidrug resistance (MDR) represents a serious problem in cancer treatment. One strategy to overcome this obstacle is to identify agents that are selectively lethal to MDR cells. The aim of this study was to discover novel compounds against MDR leukemia and to determine the molecular mechanisms behind collateral sens...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/j.bcp.2017.10.008

    authors: Hamdoun S,Fleischer E,Klinger A,Efferth T

    更新日期:2017-12-15 00:00:00

  • 9-(2-Phosphonylmethoxyethyl)-N6-cyclopropyl-2,6-diaminopurine (cpr-PMEDAP) as a prodrug of 9-(2-phosphonylmethoxyethyl)guanine (PMEG).

    abstract::9-(2-Phosphonylmethoxyethyl)-N6-cyclopropyl-2,6-diaminopurine (cpr-PMEDAP) is an acyclic nucleotide analog of the [9-(2-phosphonylmethoxyethyl)-] (PME) series containing a cyclopropyl substituent on the N6 position of the 2,6-diaminopurine (DAP) base. Growth inhibition assays in a broad range of tumor cell lines demon...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/s0006-2952(99)00138-0

    authors: Compton ML,Toole JJ,Paborsky LR

    更新日期:1999-08-15 00:00:00

  • Saxifragifolin D induces the interplay between apoptosis and autophagy in breast cancer cells through ROS-dependent endoplasmic reticulum stress.

    abstract::Breast cancer is the leading cause of cancer death among females, and novel chemotherapeutic drugs for treating breast cancer are needed urgently. Saxifragifolin D (SD) was isolated by our group from Androsace umbellata which is commonly used to treat solid tumor. In this study, we evaluated its growth inhibitory effe...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/j.bcp.2013.01.009

    authors: Shi JM,Bai LL,Zhang DM,Yiu A,Yin ZQ,Han WL,Liu JS,Li Y,Fu DY,Ye WC

    更新日期:2013-04-01 00:00:00

  • Accumulation of gamma-globin mRNA and induction of irreversible erythroid differentiation after treatment of CML cell line K562 with new doxorubicin derivatives.

    abstract::Human chronic myelogenous leukemia (CML) cell line K562 can be chemically induced to differentiate and express embryonic and fetal globin genes. In this study, the effects of doxorubicin (DOX), an inducer of K562 cell erythroid differentiation, with those of epidoxorubicin (EDOX) as well as newly synthesized derivativ...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/j.bcp.2006.09.028

    authors: Szulawska A,Arkusinska J,Czyz M

    更新日期:2007-01-15 00:00:00

  • Modifications of oxido-reductase activities in adriamycin-resistant leukaemia K562 cells.

    abstract::Adriamycin (ADR), a well-known antitumoral drug, interacts with DNA (nuclear and mitochondrial) and cardiolipin. Moreover, ADR induces numerous mitochondrial modifications in sensitive cells. However, no results have yet been obtained as to the repercussions of drug effects on oxido-reductase activities in ADR-resista...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/s0006-2952(98)00084-7

    authors: Denis-Gay M,Petit JM,Mazat JP,Ratinaud MH

    更新日期:1998-08-15 00:00:00

  • Developmental correlation of higher levels of estrogen binding by macromolecules in rat liver supernatant and of increases in plasma renin substrate levels after estrogen administration.

    abstract::Developmental correlation of higher levels of estrogen binding by macromolecules in rat liver supernatant and of increases in plasma renin substrate levels after estrogen administration is reported. Gel filtration columns were used to separate bound from free radioactivity in studying binding of radioactive estradiol...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/0006-2952(77)90467-1

    authors: Eisenfeld AJ,Krakoff LR,Aten RF

    更新日期:1977-05-15 00:00:00

  • Suppression of NF-κB signaling by andrographolide with a novel mechanism in human platelets: regulatory roles of the p38 MAPK-hydroxyl radical-ERK2 cascade.

    abstract::Andrographolide, a novel nuclear factor-κB (NF-κB) inhibitor, is isolated from leaves of Andrographis paniculata. Platelet activation is relevant to a variety of coronary heart diseases. Our recent studies revealed that andrographolide possesses potent antiplatelet activity by activating the endothelial nitric oxide s...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/j.bcp.2012.06.030

    authors: Lu WJ,Lin KH,Hsu MJ,Chou DS,Hsiao G,Sheu JR

    更新日期:2012-10-01 00:00:00

  • Deconjugating activity for sulfoconjugated dopamine in homogenates of organs from dogs.

    abstract::To clarify the possibility that sulfoconjugated dopamine (DA) may play a physiological role by being converted to active free DA, we examined the deconjugating activity in homogenates of organs from dogs. Each tissue homogenate was incubated with sulfoconjugated DA, and the deconjugating activity of the organs was com...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/0006-2952(92)90356-n

    authors: Yoshizumi M,Ohuchi T,Masuda Y,Katoh I,Oka M

    更新日期:1992-12-01 00:00:00

  • Effect of various amounts of selenium on the metabolism of mercuric chloride in mice.

    abstract::Male ddY mice were given one injection of (1) mercury (mercuric chloride) simultaneously with various doses of selenium (sodium selenite), (2) mercury alone, or (3) various doses of selenium alone. The interaction between mercury and selenium in the liver and kidneys at 1, 5, 24, 120, and 240 hr after administration w...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/0006-2952(85)90572-6

    authors: Yamamoto I

    更新日期:1985-08-01 00:00:00

  • Inhibitory effect of ionized free intracellular calcium enhanced by ruthenium red and m-chloro-carbonylcyanide phenyl hydrazon on the evoked release of acetylcholine.

    abstract::In order to understand the relationship between the free ionized calcium concentration in the axon terminals and the transmitter release we have investigated the effect of ruthenium red (RuR) and m-chloro-carbonylcyanide phenyl hydrazon (CCCP), mitochondrial uncoupler agents on the liberation of acetylcholine from mye...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/0006-2952(87)90020-7

    authors: Bernath S,Vizi ES

    更新日期:1987-11-01 00:00:00

  • BAFF activates Erk1/2 promoting cell proliferation and survival by Ca2+-CaMKII-dependent inhibition of PP2A in normal and neoplastic B-lymphoid cells.

    abstract::B-cell activating factor (BAFF) is involved in not only the physiology of normal B cells, but also the pathophysiology of aggressive B cells related to malignant and autoimmune diseases. However, how excessive BAFF promotes aggressive B-cell proliferation and survival is not well understood. Here we show that excessiv...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/j.bcp.2013.11.006

    authors: Liang D,Zeng Q,Xu Z,Zhang H,Gui L,Xu C,Chen S,Zhang S,Huang S,Chen L

    更新日期:2014-01-15 00:00:00

  • Differentiation of human hepatoma cells during confluence as revealed by gene expression profiling.

    abstract::Certain human hepatocarcinoma cells undergo differentiation when grown at confluence. In order to understand the basis for this differentiation, we investigated the phenotypic changes occurring during confluent growth of the human hepatoma B16A2 cell line. The global gene expression profile of B16A2 cells grown during...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/j.bcp.2003.10.033

    authors: Butura A,Johansson I,Nilsson K,Wärngård L,Ingelman-Sundberg M,Schuppe-Koistinen I

    更新日期:2004-04-01 00:00:00

  • Cellular pharmacology of deferrioxamine B and derivatives in cultured rat hepatocytes in relation to iron mobilization.

    abstract::Two radiolabelled derivatives of deferrioxamine B (DF) have been synthesized: methyl-DF and acetyl-DF. Both derivatives are non cytotoxic and stable in cell culture but they are degraded in human plasma and more extensively in rat plasma. Methyl-DF, acetyl-DF and DF mobilize radioiron to the same extent from hepatocyt...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/0006-2952(85)90492-7

    authors: Laub R,Schneider YJ,Octave JN,Trouet A,Crichton RR

    更新日期:1985-04-15 00:00:00

  • Regional distribution of calcium channel ligand (1,4-dihydropyridine) binding sites and 45Ca2+ uptake processes in rat brain.

    abstract::The binding of nimodipine, a 1,4-dihydropyridine Ca2+ channel antagonist, and of Bay K 8644, a Ca2+ channel activator, was measured in several regions of rat brain and compared to the distribution of K+ depolarization-induced 45Ca2+ uptake into synaptosomes. The maximum binding densities (Bmax) of [3H]nimodipine and [...

    journal_title:Biochemical pharmacology

    pub_type: 杂志文章

    doi:10.1016/0006-2952(87)90575-2

    authors: Skattebøl A,Triggle DJ

    更新日期:1987-12-01 00:00:00