当前位置： SCI文献检索 > ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS期刊下所有文献 > Functional characterization of the carnitine transporter defective in primary carnitine deficiency.

Functional characterization of the carnitine transporter defective in primary carnitine deficiency.

Abstract：

:Primary carnitine deficiency is an autosomal recessive disorder caused by defective carnitine transport which impairs fatty acid oxidation and manifests as nonketotic hypoglycemia or skeletal or heart myopathy. Here we report the functional characterization of this transporter in human fibroblasts. Carnitine enters normal cells by saturable and unsaturable routes, the latter corresponding to Na+-independent uptake. Saturable carnitine transport was absent in cells from patients with primary carnitine deficiency. In control cells, saturable carnitine transport was energized by the electrochemical gradient of Na+. Carnitine uptake was not inhibited by amino acid substrates of transport systems A, ASC, and X-AG, but was inhibited competitively (in potency order) by butyrobetaine > carnitine > palmitoylcarnitine = acetylcarnitine > betaine. Carnitine uptake was also noncompetitively inhibited by verapamil and quinidine, inhibitors of the multidrug resistance family of membrane transporters, suggesting that the carnitine transporter may share a functional motif with this class of transporters. A high-affinity carnitine transporter was present in kidney 293 cells, but not in HepG2 liver cells, whose carnitine transporter had a Km in the millimolar range. These result indicate the presence of multiple types of carnitine transporters in human cells.

journal_name

Arch Biochem Biophys

journal_title

Archives of biochemistry and biophysics

authors

Scaglia F,Wang Y,Longo N

doi

10.1006/abbi.1999.1118

subject

Has Abstract

pub_date

1999-04-01 00:00:00

pages

99-106

issue

1

eissn

0003-9861

issn

1096-0384

pii

S0003-9861(99)91118-9

journal_volume

364

pub_type

杂志文章

相关文献

ARCHIVES OF BIOCHEMISTRY AND BIOPHYSICS文献大全
  • Differential binding properties of human pregnancy zone protein- and alpha2-macroglobulin-proteinase complexes to low-density lipoprotein receptor-related protein.

    abstract：:Human pregnancy zone protein (PZP) is a major pregnancy-associated plasma protein strongly related to alpha2-macroglobulin (alpha2-M). Both alpha-macroglobulins (alpha-Ms) covalently bind proteinases, which is accompanied by the exposure of carboxy terminal receptor recognition domains important for the rapid clearanc...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1006/abbi.2001.2659

    authors： Chiabrando GA,Vides MA,Sánchez MC

    更新日期：2002-02-01 00:00:00

  • Stability of the complex formed between French bean (Phaseolus vulgaris) phenylalanine ammonia-lyase and its transition-state analog.

    abstract：:Phenylalanine ammonia-lyase forms trans-cinnamate from L-phenylalanine, and thus stands at a gateway to secondary metabolism in higher plants. L-alpha-Amino-oxy-beta-phenylpropanoic acid (L-AOPP), a very effective competitive inhibitor of this enzyme, is most probably a transition-state analog for the elimination reac...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/0003-9861(84)90265-0

    authors： Jones DH,Northcote DH

    更新日期：1984-11-15 00:00:00

  • Kinetic and spectroscopic characterization of the putative monooxygenase domain of human MICAL-1.

    abstract：:MICALs form a conserved multidomain protein family essential for cytoskeletal rearrangements. To complement structural information available, we produced the FAD-containing monooxygenase-like domain of human MICAL-1 (MICAL-MO) in forms differing for the presence and location of a His-tag, which only influences the pro...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/j.abb.2011.08.004

    authors： Zucchini D,Caprini G,Pasterkamp RJ,Tedeschi G,Vanoni MA

    更新日期：2011-11-01 00:00:00

  • 1H-NMR spectroscopic manifestations of ligand binding to the kringle 4 domain of human plasminogen.

    abstract：:Structural aspects of the binding of the linear ligands N alpha-acetyl-L-lysine (AcLys) and epsilon-aminocaproic acid (epsilon ACA) and of the cyclic analogs trans-(aminomethyl)-cyclohexanecarboxylic acid (AMCHA) and p-benzylaminesulfonic acid (BASA) to the intact plasminogen kringle 4 domain have been investigated by...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/0003-9861(86)90642-9

    authors： De Marco A,Laursen RA,Llinas M

    更新日期：1986-02-01 00:00:00

  • Regulation of 25-hydroxyvitamin D3 1alpha-hydroxylase gene expression by parathyroid hormone and 1,25-dihydroxyvitamin D3.

    abstract：:The conversion of 25-hydroxyvitamin D3 to 1,25-dihydroxyvitamin D3 (1,25-(OH)2D3) takes place mainly in the kidney and is catalyzed by the enzyme 1alpha-hydroxylase. Parathyroid hormone (PTH) and 1,25-(OH)2D3 are well-known regulators of this tightly controlled step, but the mechanisms by which they modulate 1alpha-hy...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1006/abbi.2000.1970

    authors： Brenza HL,DeLuca HF

    更新日期：2000-09-01 00:00:00

  • Rat liver glucose-6-phosphatase system: light scattering and chemical characterization.

    abstract：:Glucose-6-phosphatase is a multicomponent system located in the endoplasmic reticulum, involving both a catalytic subunit (G6PC) and several substrate and product carriers. The glucose-6-phosphate carrier is called G6PT1. Using light scattering, we determined K(D) values for phosphate and glucose transport in rat live...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/s0003-9861(02)00523-4

    authors： Clottes E,Middleditch C,Burchell A

    更新日期：2002-12-01 00:00:00

  • Expression of UDP-glucuronosyltransferases (UGTs) 2B7 and 1A6 in the human brain and identification of 5-hydroxytryptamine as a substrate.

    abstract：:The extrahepatic expression of UDP-glucuronosyltransferases (UGTs) is important in the detoxification of a number of endogenous and exogenous compounds, including 5-hydroxytryptamine and morphine. Studies were designed to investigate the extrahepatic expression of human UGTs using RT-PCR techniques and to determine th...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1006/abbi.1999.1155

    authors： King CD,Rios GR,Assouline JA,Tephly TR

    更新日期：1999-05-01 00:00:00

  • Complex, unusual conformational changes in kidney betaine aldehyde dehydrogenase suggested by chemical modification with disulfiram.

    abstract：:The NAD+-dependent animal betaine aldehyde dehydrogenases participate in the biosynthesis of glycine betaine and carnitine, as well as in polyamines catabolism. We studied the kinetics of inactivation of the porcine kidney enzyme (pkBADH) by the drug disulfiram, a thiol-reagent, with the double aim of exploring the en...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/j.abb.2007.09.022

    authors： Ayala-Castro HG,Valenzuela-Soto EM,Figueroa-Soto CG,Muñoz-Clares RA

    更新日期：2007-12-15 00:00:00

  • Difference in polymerization and steady-state dynamics of free and gelsolin-capped filaments formed by alpha- and beta-isoactins.

    abstract：:The polymerization of scallop beta-like actin is significantly slower than that of skeletal muscle alpha-actin. To reveal which steps of polymerization contribute to this difference, we estimated the efficiency of nucleation of the two actins, the rates of filament elongation at spontaneous and gelsolin-nucleated poly...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/j.abb.2008.06.016

    authors： Khaitlina S,Hinssen H

    更新日期：2008-09-15 00:00:00

  • Development of a peptide antibody specific to human glutathione S-transferase alpha 4-4 (hGSTA4-4) reveals preferential localization in human liver mitochondria.

    abstract：:The reactive cellular products generated during the peroxidation of membrane lipids have been implicated as causative agents in a variety of degenerative diseases and aging. In particular, 4-hydroxynon-2-enal (4HNE) is among the most of the produced during lipid peroxidation. In humans and rodent species, the alpha 4 ...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1006/abbi.2001.2352

    authors： Gardner JL,Gallagher EP

    更新日期：2001-06-01 00:00:00

  • Cross-linking of egg white riboflavin-binding protein by calcium phosphate.

    abstract：:No larger molecular weight component appeared in the high-performance gel chromatogram when calcium alone was added to the riboflavin-binding protein solution (RfBP), indicating that calcium alone did not aggregate it. RfBP bound calcium, but the amount of bound calcium decreased markedly upon dephosphorylation. Cross...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1006/abbi.1993.1417

    authors： Aoki T,Yamao Y,Yonemasu E,Kumasaki Y,Kako Y

    更新日期：1993-09-01 00:00:00

  • Secondary structure and calcium-induced folding of the Clostridium thermocellum dockerin domain determined by NMR spectroscopy.

    abstract：:Assembly of the cellulosome, a large, extracellular cellulase complex, depends upon docking of a myriad of enzymatic subunits to homologous receptors, or cohesin domains, arranged in tandem along a noncatalytic scaffolding protein. Docking to the cohesin domains is mediated by a highly conserved domain, dockerin (DS),...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1006/abbi.2000.1882

    authors： Lytle BL,Volkman BF,Westler WM,Wu JH

    更新日期：2000-07-15 00:00:00

  • Physicochemical and saccharide-binding studies on the galactose-specific seed lectin from Trichosanthes cucumerina.

    abstract：:Physicochemical and saccharide-binding studies have been performed on Trichosanthes cucumerina seed lectin (TCSL). The agglutination activity of TCSL is highest in the pH range 8.0-11.0, whereas below pH 7.0 it decreases quite rapidly, which is consistent with the involvement of imidazole side chains of His residues, ...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/s0003-9861(03)00094-8

    authors： Kenoth R,Komath SS,Swamy MJ

    更新日期：2003-05-01 00:00:00

  • Effect of genetic polymorphism on the inhibition of dopamine formation from p-tyramine catalyzed by brain cytochrome P450 2D6.

    abstract：:The inhibitory effects of steroid hormones, including glucocorticoids such as cortisol, and related compounds on dopamine formation from p-tyramine, catalyzed by cytochrome P450 (CYP) 2D6.2 (Arg296Cys, Ser486Thr) and CYP2D6.10 (Pro34Ser, Ser486Thr) were compared with the effects of those catalyzed by CYP2D6.1 (wild ty...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/j.abb.2017.03.009

    authors： Niwa T,Shizuku M,Yamano K

    更新日期：2017-04-15 00:00:00

  • L-carnitine delays the killing of cultured hepatocytes by 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine.

    abstract：:The role of fatty acid metabolism in chemical-dependent cell injury is poorly understood. Addition of L-carnitine to the incubation medium of cultured hepatocytes delayed cell killing initiated by 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP). Protection by L-carnitine was stereospecific and observed as late as ...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/0003-9861(90)90019-u

    authors： Snyder JW,Kyle ME,Ferraro TN

    更新日期：1990-01-01 00:00:00

  • Properties and subcellular localization of adenosine diphosphatase in rat heart.

    abstract：:Some properties and subcellular localization of adenosine diphosphatase (ADPase) activity from rat heart have been investigated. The pH optimum was 7.4, maximal activity was found with 5 mM MgCl2, and the apparent Km was 20 microM. ADPase activity was strongly inhibited by NaF and AppNHp, and to a lesser extent by AMP...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/0003-9861(84)90615-5

    authors： de Vente J,Velema J,Zaagsma J

    更新日期：1984-08-15 00:00:00

  • Similarities between the Mr 245,000 calmodulin-binding protein of the dogfish erythrocyte cytoskeleton and alpha-fodrin.

    abstract：:The Mr 245,000 calmodulin-binding protein of the dogfish erythrocyte cytoskeleton (D245) has been compared with human erythrocyte spectrin and mammalian brain fodrin [J. Levine and M. Willard (1981) J. Cell Biol. 90, 631-643]. Mammalian erythrocyte alpha-spectrin, brain alpha-fodrin, and D245 are all localized in the ...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/0003-9861(84)90081-x

    authors： Bartelt DC,Carlin RK,Scheele GA,Cohen WD

    更新日期：1984-04-01 00:00:00

  • Purification, physicochemical characterization, saccharide specificity, and chemical modification of a Gal/GalNAc specific lectin from the seeds of Trichosanthes dioica.

    abstract：:A new galactose-specific lectin has been purified from the extracts of Trichosanthes dioica seeds by affinity chromatography on cross-linked guar gum. The purified lectin (T. dioica seed lectin, TDSL) moved as a single symmetrical peak on gel filtration on Superose-12 in the presence of 0.1 M lactose with an M(r) of 5...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/j.abb.2004.09.016

    authors： Sultan NA,Kenoth R,Swamy MJ

    更新日期：2004-12-15 00:00:00

  • CoA recycling by a benzoate coenzyme A ligase in cascade reactions with aroyltransferases to biocatalyze paclitaxel analogs.

    abstract：:A Pseudomonas CoA ligase (BadA) biocatalyzed aroyl CoA thioesters used by a downstream N-benzoyltransferase (NDTNBT) in a cascade reaction made aroyl analogs of the anticancer drug paclitaxel. BadA kept the high-cost aroyl CoA substrates at saturation for the downstream NDTNBT by recycling CoA when it was added as the...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/j.abb.2020.108276

    authors： Sullivan SA,Nawarathne IN,Walker KD

    更新日期：2020-04-15 00:00:00

  • Topoisomerase IIβ associates with Ku70 and PARP-1 during double strand break repair of DNA in neurons.

    abstract：:In the present study, the activity of Topoisomerase IIβ (TopoIIβ) is evaluated during peroxide induced double stranded DNA breaks (DSBs) repair in primary neurons. The results showed that the TopoIIβ levels were enhanced during recovery from peroxide mediated damage (PED) along with Ku70, PARP-1, pol beta, and WRN hel...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/j.abb.2011.10.001

    authors： Mandraju R,Chekuri A,Bhaskar C,Duning K,Kremerskothen J,Kondapi AK

    更新日期：2011-12-15 00:00:00

  • Transformation of an arachidonic acid hydroperoxide into epoxyhydroxy and trihydroxy fatty acids by liver microsomal cytochrome P-450.

    abstract：:In the absence of NADPH, the addition of an arachidonic acid hydroperoxide, 15-hydroperoxyeicosa-5,8,11,13-tetraenoic acid, to liver microsomes, prepared from phenobarbital-treated rats, resulted in the formation of two major metabolites and several minor products, some of which have been purified by reverse-phase hig...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/0003-9861(87)90039-7

    authors： Weiss RH,Arnold JL,Estabrook RW

    更新日期：1987-01-01 00:00:00

  • A conserved hydrophobic core at Bcl-xL mediates its structural stability and binding affinity with BH3-domain peptide of pro-apoptotic protein.

    abstract：:Bcl-2 family proteins regulate apoptosis through their homo- and heterodimerization. By protein sequence analysis and structural comparison, we have identified a conserved hydrophobic core at the BH1 and BH2 domains of Bcl-2 family proteins. The hydrophobic core is stabilized by hydrophobic interactions among the resi...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/j.abb.2009.01.003

    authors： Feng Y,Zhang L,Hu T,Shen X,Ding J,Chen K,Jiang H,Liu D

    更新日期：2009-04-01 00:00:00

  • Amino acid sequences of two high-potential iron-sulfur proteins (HiPIPs) from the moderately halophilic purple phototrophic bacterium, Rhodospirillum salinarum.

    abstract：:The amino acid sequences of two very different high-potential iron-sulfur protein (HiPIP) isozymes have been determined from the moderately halophilic purple phototrophic bacterium, Rhodospirillum salinarum. Iso-1 HiPIP, which is monomeric and contains 57 amino acid residues, is most similar to the Thiobacillus ferroo...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1006/abbi.1999.1323

    authors： Ambler RP,Daniel M,Meyer TE,Cusanovich MA

    更新日期：1999-09-01 00:00:00

  • Effect of ethanol on tropomyosin in solution and in reconstituted thin filaments.

    abstract：:The effects of ethanol at concentrations below 10% on the conformation of tropomyosin, its end-to-end polymerization, its binding to F-actin, and its effects on actomyosin ATPase activity were studied. Ethanol stabilized the tropomyosin conformation by shifting the helix thermal unfolding profile to higher temperature...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/0003-9861(86)90333-4

    authors： Ishii Y,Lehrer SS,Ohnishi ST,Rubin E

    更新日期：1986-05-01 00:00:00

  • Effect of age and dietary calcium on intestinal calbindin D-9k expression in the rat.

    abstract：:The capacity of rats and humans to adapt to low dietary Ca by increasing intestinal Ca absorption declines with age. The intestinal calbindin-D-9k protein (calbindin) is thought to play a role in the transcellular transport of Ca across the mammalian intestine. The purpose of these studies was to determine the effect ...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/j.abb.2003.09.025

    authors： Armbrecht HJ,Boltz MA,Bruns ME

    更新日期：2003-12-01 00:00:00

  • Cloning and characterization of the mouse cytochrome c oxidase subunit IV gene.

    abstract：:cDNA for mouse cytochrome c oxidase subunit IV (COXIV) was isolated by screening mouse liver and kidney cDNA libraries with a bovine COXIV cDNA probe. The 679-nucleotide nearly full length cDNA codes for a 22-amino acid presequence and a 147-amino acid mature protein which show 77 to 95% positional identity with the p...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/0003-9861(91)90169-j

    authors： Carter RS,Avadhani NG

    更新日期：1991-07-01 00:00:00

  • Stimulation of bovine brain phospholipase C activity by myelin basic protein requires arginyl residues in peptide linkage.

    abstract：:We reported previously a highly purified phosphatidylinositol-specific phospholipase C (PI-PLC) from bovine brain and from human myelin which was stimulated by myelin basic protein. In this paper we report that the stimulation of the PI-PLC activity by myelin basic protein (MBP) requires arginine residues in peptide l...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1006/abbi.1993.1242

    authors： Tompkins TA,Moscarello MA

    更新日期：1993-05-01 00:00:00

  • Carbon-centered free radical intermediates in the hematin- and ram seminal vesicle-catalyzed decomposition of fatty acid hydroperoxides.

    abstract：:Heme-catalyzed decomposition of unsaturated hydroperoxy fatty acids has been proposed to proceed via carbon-centered free radicals (delocalized at positions C11, C12, and C13 for 15-hydroperoxy-eicosatetraenoic acid (15-HPETE). The stable products are usually epoxy fatty acids and epoxy alcohols. Hydroperoxides from a...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/0003-9861(86)90046-9

    authors： Schreiber J,Mason RP,Eling TE

    更新日期：1986-11-15 00:00:00

  • Translational repression by chemical inducers of the stress response occurs by different pathways.

    abstract：:The mechanism by which chemical inducers of the stress response inhibit protein synthesis was examined. All the chemicals tested principally inhibit the initiation phase of translation. Covalent modification of the initiation factor proteins does not constitute a common mechanism. Eukaryotic initiation factor (eIF)-2 ...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/0003-9861(87)90622-9

    authors： Duncan RF,Hershey JW

    更新日期：1987-08-01 00:00:00

  • Synthesis and accumulation of an extremely stable high-energy phosphate compound by muscle, heart, and brain of animals fed the creatine analog, 1-carboxyethyl-2-iminoimidazolidine (homocyclocreatine).

    abstract：:A new creatine analog, 1-carboxyethyl-2-iminoimidazolidine (homocyclocreatine), has been synthesized and compared with other synthetic analogs of creatine as a substrate for creatine kinase under both in vitro and in vivo conditions. Reactivity with rabbit muscle creatine kinase at 2 mM and pH 7.0 occurred in the orde...

    journal_title：Archives of biochemistry and biophysics

    pub_type： 杂志文章

    doi：10.1016/0003-9861(83)90448-4

    authors： Roberts JJ,Walker JB

    更新日期：1983-02-01 00:00:00