Evidence for a mitochondrial lesion in cystic fibrosis.

Abstract:

:Cystic fibrosis (CF) remains a major problem in human genetics and cell pathophysiology. It is a single gene trait caused by a mutation on the long arm of chromosome 7. Among its expressions are abnormal regulation of chloride channels and/or microobstructions in exocrine tissues. Here, evidence is presented that mitochondria are dysfunctional in CF: the major site of increased intracellular Ca in CF is mitochondrial, cells from subjects with CF consume more oxygen than normal, respond differentially to inhibitors of mitochondrial function, express increased electron transport activity and altered kinetics of complex I (NADH dehydrogenase) of the mitochondrial electron transport system. Patients with CF express increased total and resting energy expenditure. Some of these differences from normal occur also in asymptomatic carriers of the CF gene.

journal_name

Life Sci

journal_title

Life sciences

authors

Shapiro BL

doi

10.1016/0024-3205(89)90389-5

subject

Has Abstract

pub_date

1989-01-01 00:00:00

pages

1327-34

issue

19

eissn

0024-3205

issn

1879-0631

pii

0024-3205(89)90389-5

journal_volume

44

pub_type

杂志文章,评审
  • Effect of 7-(3-[4-(2,3-dimethylphenyl)piperazinyl]propoxy)- 2(1H)-quinolinone (OPC-4392), a newly synthesized agonist for presynaptic dopamine D2 receptor, on tyrosine hydroxylation in rat striatal slices.

    abstract::The effects of a newly synthesized compound, 7-(3-[4-(2,3-dimethylphenyl)piperazinyl]propoxy)-2(1H)-quinolinone (OPC-4392), on tyrosine hydroxylation in situ and in vitro were studied using rat striatal slices and tyrosine hydroxylase (TH) purified from bovine adrenal medulla, respectively. OPC-4392 dose-dependently i...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(88)90644-3

    authors: Kiuchi K,Hirata Y,Minami M,Nagatsu T

    更新日期:1988-01-01 00:00:00

  • Hyperforin inhibits vesicular uptake of monoamines by dissipating pH gradient across synaptic vesicle membrane.

    abstract::Extracts of Hypericum perforatum (St. John's wort) have antidepressant properties in depressed patients and exert antidepressant-like action in laboratory animals. The phloroglucinol derivative hyperforin has become a topic of interest, as this Hypericum component is a potent inhibitor of monoamines reuptake. The mole...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/s0024-3205(03)00295-9

    authors: Roz N,Rehavi M

    更新日期:2003-06-13 00:00:00

  • Highly-sensitive identification of alpha-fetoprotein mRNA in circulating peripheral blood of hepatocellular carcinoma patients.

    abstract::In order to capture hepatocellular carcinoma (HCC) cells in circulating peripheral blood, we made analysis to see if alpha-fetoprotein (AFP) mRNA exists in the peripheral blood obtained from patients with HCC and also, as a control, from hepatitis-viral-marker-positive patients without HCC and a healthy volunteer. As ...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(95)02138-9

    authors: Funaki N,Tanaka J,Seto S,Kasamatsu T,Kaido T,Imamura M

    更新日期:1995-01-01 00:00:00

  • High glucose increases platelet-derived growth factor production in cultured human vascular endothelial cells and preventive effects of eicosapentaenoic acids.

    abstract::The regulation of the production of platelet-derived growth factor (PDGF) and the influence of high glucose concentration, eicosapentaenoic acid (EPA) were studied in cultured human umbilical vein endothelial cells (HUE). The PDGF production of HUE increased markedly depending on glucose concentration. However, EPA (3...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(95)00261-4

    authors: Mizutani M,Okuda Y,Suzuki S,Sawada T,Soma M,Yamashita K

    更新日期:1995-01-01 00:00:00

  • Acid-induced modulation of airway basal tone and contractility: role of acid-sensing ion channels (ASICs) and TRPV1 receptor.

    abstract::The role of extracellular acidosis in inflammatory airway diseases is not well known. One consequence of tissue acidification is the stimulation of sensory nerves via the polymodal H(+)-gated transmembrane channels ASICs and TRPV1 receptor. The present study investigated the effect of acidosis on airway basal tone and...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/j.lfs.2007.08.026

    authors: Faisy C,Planquette B,Naline E,Risse PA,Frossard N,Fagon JY,Advenier C,Devillier P

    更新日期:2007-09-08 00:00:00

  • Role of superoxide radicals in anoxia reoxygenation-mediated vascular contraction.

    abstract::To determine the mechanism of anoxic vasoconstriction, precontracted rat aortic rings were exposed to 95% N2, which caused additional contraction. Reoxygenation (95% O2) resulted in initial relaxation followed by contraction. Indomethacin did not affect anoxic contraction or reoxygenation-mediated events, but NG-monom...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(91)90316-4

    authors: Mehta JL,Lawson DL,Yang BC,Haught WH,Hintze T

    更新日期:1991-01-01 00:00:00

  • Supplementation of L-arginine improves hypertension and lipid metabolism but not insulin resistance in diabetic rats.

    abstract::Nitric oxide (NO) plays an important role in glucose and lipid metabolism. We previously reported that NO synthesis inhibitors, such as NG-nitro-L-arginine methyl ester (L-NAME), deteriorate insulin sensitivity and lipid metabolism, while the addition of L-arginine reverses this deterioration. L-arginine is a precurso...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/j.lfs.2003.06.004

    authors: Kawano T,Nomura M,Nisikado A,Nakaya Y,Ito S

    更新日期:2003-10-24 00:00:00

  • Ethanol and caffeine consumption modulates the expression of miRNAs in the cerebellum and plasma of UChB rats.

    abstract:AIMS:The present study aimed to verify changes in cerebellar and plasmatic expression of miRNAs after the chronic consumption of ethanol and caffeine in the UChB rat, an experimental model for alcoholism. MATERIAL AND METHODS:Male rats at 5 months of age, were divided into the following groups (n = 10/group): 1. Ethan...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/j.lfs.2019.05.016

    authors: Rossetto IMU,Cagnon VHA,Lizarte FSN,Tirapelli LF,Tirapelli DPC,Arantes RMS,Chuffa LGA,Martinez FE,Martinez M

    更新日期:2019-07-15 00:00:00

  • Methyl and isopropyl N-methylanthranilates attenuate diclofenac- and ethanol-induced gastric lesions in rats.

    abstract:AIMS:Two natural alkaloids, methyl (M) and isopropyl (I) N-methylanthranilates, with recently demonstrated significant pharmacological activities, were assayed for their possible overall effect on intact gastric mucosa and their protective properties towards the onset of gastric lesions induced by diclofenac (a non-ste...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/j.lfs.2013.10.002

    authors: Radulović NS,Jovanović I,Ilić IR,Randjelović PJ,Stojanović NM,Miltojević AB

    更新日期:2013-11-19 00:00:00

  • Role of p62/SQSTM1 in lipopolysaccharide (LPS)-induced mucus hypersecretion in bronchial epithelial cells.

    abstract:AIMS:The aims of this study were to evaluate the effects of p62/SQSTM1 expression levels on lipopolysaccharide (LPS)-induced mucus secretion in BEAS-2B bronchial epithelial cells by measuring expression levels of the MUC5AC gene and the Mucin-5AC (MUC5AC) protein. MATERIALS AND METHODS:Bronchial epithelial cells, BEAS...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/j.lfs.2018.09.030

    authors: Wu Y,Li Y,Wang B,He X,Li Y,Wu B,Yu G,Wang H,Xu B

    更新日期:2018-10-15 00:00:00

  • Inhibition by N-ethylmaleimide of H(+)-ATPase reduces the cellular action of arginine vasopressin in cultured rat renal papillary collecting tubule cells.

    abstract::We examined whether H(+)-ATPase is involved in the control of cellular action of arginine vasopressin (AVP) to produce adenosine 3', 5'-monophosphate (cAMP) and mobilize cellular free calcium ([Ca2+]i) in rat renal papillary collecting tubule cells in culture. N-Ethylmaleimide (NEM), an inhibitor of H(+)-ATPase, reduc...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(93)90594-s

    authors: Ishikawa S,Sakuma N,Okada K,Saito T

    更新日期:1993-01-01 00:00:00

  • Ontogeny of endothelin and its receptors in rat brain.

    abstract::The ontogeny of endothelin (ET) system in rats was studied in preterm (18 days of gestation), term (21 days of gestation) and 1 week post term rats. Brains were dissected out and (1) processed for the estimation of endogenous ET-1 by RIA and (2) membranes were prepared for radioreceptor binding. Receptor characteristi...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(92)90300-e

    authors: Gulati A,Rebello S,Chari G,Bhat R

    更新日期:1992-01-01 00:00:00

  • Alkaline phosphatase activity during sphinganine potentiation of retinoic acid-induced differentiation of human promyelocytic leukemia cell line, HL-60.

    abstract::Sphinganine (SP) pre-treatment potentiated the retinoic acid (RA)-induced (4-96h exposures) differentiation and increase of alkaline phosphatase (ALP) activity. A higher percentage of SP pre-treated cells in RA exposures resembled mature myelocytes or granulocytes; greater increase in ALP activity was observed. In cel...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(93)90688-y

    authors: Wei LL,Hui EK,Wei JS,Tzeng WF,Yung BY

    更新日期:1993-01-01 00:00:00

  • Fast-acting excitatory amino acids are involved in the enhancement of the aversiveness of the electrical stimulation of the inferior colliculus by systemic injections of muscimol.

    abstract::Gradual increases in the electrical stimulation of the inferior colliculus produces progressive aversive responses from vigilance, through freezing, until escape. These responses are probably mediated by excitatory amino acids (EAA) mechanisms as microinjection of glutamate into the inferior colliculus can trigger fre...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/s0024-3205(02)02169-0

    authors: Nobre MJ,Borelli KG,Brandão ML

    更新日期:2002-11-08 00:00:00

  • The anti-inflammatory effects of quinolinic acid in the rat.

    abstract::Quinolinic acid (QUIN) levels are elevated in patients and animals suffering from chronic infectious diseases. In the present study, male Sprague-Dawley rats were used to test the anti-inflammatory effects of QUIN using the carrageenan (CGN)-induced paw edema assay and the CGN sponge assay. Results of these studies in...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/s0024-3205(99)00049-1

    authors: Heyliger SO,Mazzio EA,Soliman KF

    更新日期:1999-01-01 00:00:00

  • Effect of glucagon-(1-21)-peptide on secretin-stimulated pancreatic exocrine secretion in anesthetized dogs.

    abstract::The effects of glucagon-(1-21)-peptide on pancreatic exocrine secretion and plasma glucose levels were studied and compared with those of native glucagon in anesthetized dogs. Intravenous bolus administration of 1 nmol or 10 nmol/kg of glucagon-(1-21)-peptide evoked a significant inhibition of secretin-stimulated panc...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(89)90379-2

    authors: Itoh H,Matsuyama T,Namba M,Watanabe N,Komatsu R,Kono N,Tarui S

    更新日期:1989-01-01 00:00:00

  • The effect of arylazido aminopropionyl ATP on atropine resistant contractions of the cat urinary bladder.

    abstract::This work was designed to study neurogenic, atropine resistant contractions of the cat urinary bladder. Urinary bladder contractions were induced by pelvic nerve stimulation (PNS) and the administration of acetylcholine (Ach) and purine compounds. The results show that atropine (2 mg/kg) depresses the maintained porti...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(83)90374-0

    authors: Theobald RJ Jr

    更新日期:1983-05-23 00:00:00

  • The beta-carboline ZK 93423 inhibits reticulata neurons: an effect reversed by benzodiazepine antagonists.

    abstract::A novel beta-carboline with benzodiazepine-like properties has recently been synthesized. We compared the effect of the i.v. administration of this drug, ZK 93423, with diazepam on the activity of nigral pars reticulata neurons which are known to be very sensitive to the inhibitory effect produced by GABA-mimetics and...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(87)90333-x

    authors: Mereu G,Corda MG,Carcangiu P,Giorgi O,Biggio G

    更新日期:1987-04-06 00:00:00

  • Mesna (2-mercaptoethane sulfonate) prevents ischemia/reperfusion induced renal oxidative damage in rats.

    abstract::Reoxygenation of the ischemic tissue promotes the generation of various reactive oxygen metabolites (ROM) which are known to have deleterious effects on various cellular functions. This study was designed to determine the possible protective effect of mesna (2-Mercaptoethane Sulfonate) on renal ischemia/reperfusion (I...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/j.lfs.2004.04.029

    authors: Kabasakal L,Sehirli AO,Cetinel S,Cikler E,Gedik N,Sener G

    更新日期:2004-09-24 00:00:00

  • Ketanserin pretreatment attenuates MDMA-induced dopamine release in the striatum as measured by in vivo microdialysis.

    abstract::Systemic administration of the amphetamine analogue, 3,4-methylenedioxymethamphetamine (MDMA) produced a dose-dependent increase in the extracellular concentration of dopamine (DA) in the striatum as measured by in vivo microdialysis in awake, freely-moving rats. The extracellular concentration of the DA metabolite, 3...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(90)90484-9

    authors: Nash JF

    更新日期:1990-01-01 00:00:00

  • The protective effect of Hif3a RNA interference and HIF-prolyl hydroxylase inhibition on cardiomyocytes under anoxia-reoxygenation.

    abstract::The aim of this study was to investigate the molecular mechanisms underlying the protective effects of hypoxia-inducible factor (HIF) signaling pathway activation in cardiomyocytes under anoxia-reoxygenation (A/R) injury. In this study, rat neonatal cardiomyocytes were pretreated with anti-Hif3A/Hif-3α siRNA or HIF-pr...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/j.lfs.2018.04.021

    authors: Drevytska T,Gonchar E,Okhai I,Lynnyk O,Mankovska I,Klionsky D,Dosenko V

    更新日期:2018-06-01 00:00:00

  • Acute changes in growth hormone-releasing hormone secretion after injection of BIM 23014, a long acting somatostatin analog, in rams.

    abstract::BIM 23014 is a somatostatin analog displaying an increased biological half life due to resistance to enzymatic degradation. This peptide inhibits GH release directly at the level of pituitary somatotrophs. In addition, an action of BIM 23014 at the level of the hypothalamus is possible since somatostatinergic fibers a...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(92)90610-2

    authors: Magnan E,Cataldi M,Guillaume V,Conte-Devolx B,Graziani N,Figaroli JC,Thomas F,Chihara K,Oliver C

    更新日期:1992-01-01 00:00:00

  • Evolution of the activity of UGT1A1 throughout the development and adult life in a rat.

    abstract::Biliary excretion is the main route of disposal of bilirubin and impaired excretion results in jaundice, a well recognisable symptom of liver disease. Conjugation of bilirubin in the liver is essential for its clearance. The glucuronidation of bilirubin is catalysed by the microsomal UDP-glucuronosyltransferase UGT1A1...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/j.lfs.2005.08.018

    authors: Bustamante N,Cantarino MH,Arahuetes RM,Cubero FJ,Ortiz A

    更新日期:2006-03-06 00:00:00

  • High glucose-induced ROS accumulation is a critical regulator of ERK1/2-Akt-tuberin-mTOR signalling in RGC-5 cells.

    abstract::Hyperglycemia and oxidative stress are the primary stressors that elicit mitochondria specific cell stress in diabetes. Here we hypothesized that elevated level of ROS in high glucose (HG) environment, trigger mitochondrial stress by damaging mitochondrial DNA (mtDNA), altering inflammatory mediators, and neurodegener...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/j.lfs.2020.117914

    authors: Pal S,Rao GN,Pal A

    更新日期:2020-09-01 00:00:00

  • Purification and characterization of enkephalinase, angiotensin converting enzyme, and a third peptidyldipeptidase from rat brain.

    abstract::Three distinct peptidyldipeptidases (exopeptidases releasing carboxyl terminal dipeptide residues) can be solubilized from nerve terminal membrane fractions from whole rat brain or striatum, and separated by ion exchange chromatography. Brain angiotensin-converting enzyme (PDP-1) cleaves Hip-His-Leu, but not 80 nM [3H...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(83)90435-6

    authors: Cushman DW,Gordon EM,Wang FL,Cheung HS,Tung R,Delaney NG

    更新日期:1983-01-01 00:00:00

  • Co-regulation of mucosal prostanoids and substance P by indomethacin in rat stomachs.

    abstract::The present investigation examined the correlation between the regulation of mucosal prostaglandin (PG) biosynthesis and the release of the neuropeptide substance P (SP) in rat stomachs by indomethacin, a cyclooxygenase inhibitor. When given subcutaneously at the dose of 5 mg/kg, indomethacin reduced mucosal biosynthe...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/s0024-3205(97)00117-3

    authors: Ko JK,Tang F,Cho CH

    更新日期:1997-01-01 00:00:00

  • Effects of MFHAS1 on cognitive impairment and dendritic pathology in the hippocampus of septic rats.

    abstract:AIMS:To investigate the effects of malignant fibrous histiocytoma amplified sequence 1 (MFHAS1) on cognitive dysfunction, the expression of tumor necrosis factor-α (TNF-α), interleukin (IL)-1β and amyloid β peptide (Aβ) in the hippocampus, as well as dendritic pathology in the hippocampal CA1 region in sepsis-associate...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/j.lfs.2019.116822

    authors: Zhong J,Guo C,Hou W,Shen N,Miao C

    更新日期:2019-10-15 00:00:00

  • Erythrocyte 3H-ouabain binding and digitalis treatment in ethanol addicted patients.

    abstract::The binding of 3H-ouabain to human erythrocytes was analyzed in a population of hospitalized male ethanol addicted patients under long term digitalis treatment. In the non-alcoholic patient group the long term digitalis treatment induced an increase in Bmax and Kd values; such modification was not observed in the alco...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/0024-3205(87)90071-3

    authors: Battaini F,Govoni S,Mauri A,Civelli L,Trabucchi M

    更新日期:1987-06-29 00:00:00

  • Cod liver oil ameliorates sodium nitrite-induced insulin resistance and degradation of rat hepatic glycogen through inhibition of cAMP/PKA pathway.

    abstract:AIMS:Sodium nitrite is used to inhibit the growth of microorganisms and is responsible for the desirable red color of meat; however, it can be toxic in high quantities for humans and other animals. Moreover, glycogen, a branched polysaccharide, efficiently stores and releases glucose monosaccharides to be accessible fo...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/j.lfs.2014.11.002

    authors: Al-Gayyar MM,Alyoussef A,Hamdan AM,Abbas A,Darweish MM,El-Hawwary AA

    更新日期:2015-01-01 00:00:00

  • Time course sequences of angiotensin converting enzyme and chymase-like activities during development of right ventricular hypertrophy induced by pulmonary artery constriction in dogs.

    abstract::ACE and chymase play crucial roles in the establishment of pressure overload-induced cardiac hypertrophy. In the present study, time sequences of ACE and chymase-like activities, and their correlation with hypertrophic changes including free wall thickness and cardiac fibrosis, were elucidated in dogs with constant pr...

    journal_title:Life sciences

    pub_type: 杂志文章

    doi:10.1016/j.lfs.2004.04.006

    authors: Orito K,Yamane T,Kanai T,Fujii Y,Wakao Y,Matsuda H

    更新日期:2004-07-16 00:00:00