Abstract：
:Cardiomyopathy is one of the most common causes of chronic heart failure worldwide. Mutations in the gene encoding nexilin (NEXN) occur in patients with both hypertrophic and dilated cardiomyopathy (DCM); however, little is known about the pathophysiological mechanisms and relevance of NEXN to these disorders. Here, we evaluated the functional role of NEXN using a constitutive Nexn knock-out (KO) mouse model. Heterozygous (Het) mice were inter-crossed to produce wild-type (WT), Het, and homozygous KO mice. At birth, 32, 46, and 22 % of the mice were WT, Het, and KO, respectively, which is close to the expected Mendelian ratio. After postnatal day 6, the survival of the Nexn KO mice decreased dramatically and all of the animals died by day 8. Phenotypic characterizations of the WT and KO mice were performed at postnatal days 1, 2, 4, and 6. At birth, the relative heart weights of the WT and KO mice were similar; however, at day 4, the relative heart weight of the KO group was 2.3-fold higher than of the WT group. In addition, the KO mice developed rapidly progressive cardiomyopathy with left ventricular dilation and wall thinning and decreased cardiac function. At day 6, the KO mice developed a fulminant DCM phenotype characterized by dilated ventricular chambers and systolic dysfunction. At this stage, collagen deposits and some elastin deposits were observed within the left ventricle cavity, which resembles the features of endomyocardial fibroelastosis (EFE). Overall, these results further emphasize the role of NEXN in DCM and suggest a novel role in EFE.
journal_name
Basic Res Cardioljournal_title
Basic research in cardiologyauthors
Aherrahrou Z,Schlossarek S,Stoelting S,Klinger M,Geertz B,Weinberger F,Kessler T,Aherrahrou R,Moreth K,Bekeredjian R,Hrabě de Angelis M,Just S,Rottbauer W,Eschenhagen T,Schunkert H,Carrier L,Erdmann Jdoi
10.1007/s00395-015-0522-5subject
Has Abstractpub_date
2016-01-01 00:00:00pages
6issue
1eissn
0300-8428issn
1435-1803pii
10.1007/s00395-015-0522-5journal_volume
111pub_type
杂志文章abstract：:Ultrasonic techniques for the measurement of ventricular dimensions are widely used in acute and chronic experiments. Implantation of ultrasonic crystals is associated with reversible and irreversible myocardial damage which might limit the interpretation of the obtained results, in particular during acute experiments...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF01907029
更新日期：1987-07-01 00:00:00
abstract：:Protection from postconditioning has been documented in in situ animal models and it has been proposed that it is targeting circulating leukocytes. We therefore tested whether postconditioning can protect leukocyte-free, buffer-perfused rabbit hearts. Infarct size was measured with triphenyltetrazolium staining. In co...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/s00395-004-0498-4
更新日期：2005-01-01 00:00:00
abstract：:Cardiac alpha- and beta-adrenoceptors and the positive inotropic effects of several adenylate cyclase dependent and independent agents have been measured in papillary muscle strips from patients without, as well as with moderate and severe heart failure. The number of beta-adrenoceptors was found to be decreased depen...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF02650352
更新日期：1989-01-01 00:00:00
abstract：:The hormone-sensitive adenylate cyclase is a multi-component system embedded in the lipid bilayer of the plasma membrane and serves as a signal transduction system for various membrane receptors. The complete system consists of various receptor molecules, which sensitize the external ligands, the effector enzyme adeny...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF01907422
更新日期：1986-01-01 00:00:00
abstract：:A method is theoretically described to test the accuracy of various lead system of the ecg. The priniciple of this method consists in the following procedure. The heart position is changed maximally in a proband by varying the position of the body and by deep inspiration and exspiration. If the lead field is parallel,...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：
更新日期：1975-01-01 00:00:00
abstract：:Beta-blockers have been shown to reduce the risk of coronary events and reduce the mortality of patients with hypertension and in those who have had a myocardial infarction. Furthermore, the evidence for long-term benefits in these two patient groups are much more convincing for beta-blockers than for other therapeuti...
journal_title：Basic research in cardiology
pub_type： 杂志文章,评审
doi：10.1007/s003950070006
更新日期：2000-01-01 00:00:00
abstract：:The authors have retracted this article [1] because of modifications in the control lanes of Figs. 2a and 8a of the COX1 blot obtained for 18-week-old rats (rotation, horizontal flipping and re-use of the control lanes for the 35-week-old rats blot). In light of the concerns raised, the conclusions drawn in this artic...
journal_title：Basic research in cardiology
pub_type： 杂志文章,撤回出版物
doi：10.1007/s00395-018-0680-3
更新日期：2018-04-18 00:00:00
abstract：:We describe diastolic properties of the myocardium in terms of stress-strain relations. In a mathematical analysis, the equation sigma = alpha(e beta.epsilon-1) of the stress-strain curve can be changed by an increase in the exponent beta or the multiplicative constant alpha. It can be experimentally shown that in hyp...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/978-3-662-11289-2_26
更新日期：1987-01-01 00:00:00
abstract：:The concept of metabolic coronary flow control provides a rationale for the close relationship of coronary flow and myocardial metabolic rate of oxygen. The concept is based on the presence of an oxygen (metabolic) sensor coupled functionally to effector mechanisms, which control vascular tone. Four modes of metabolic...
journal_title：Basic research in cardiology
pub_type： 杂志文章,评审
doi：10.1007/s00395-006-0621-4
更新日期：2006-11-01 00:00:00
abstract：:The purpose of this study was to determine the effect of acute and chronic propranolol on heart size and regional O2 supply/consumption variables in thyroxine (T4)-treated rabbit hearts. New Zealand white rabbits were given 0.5 mg/kg T4 for 3 or 16 days with and without concomitant 2 mg/kg propranolol. Another group w...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF01907360
更新日期：1988-05-01 00:00:00
abstract：:Vasoconstrictory and vasodilatory hormone systems may be important in the regulation of peripheral vascular resistance and renal hemodynamics in the early phase of heart failure. The activity of the renin-angiotensin-aldosterone system (RAAS), the sympathetic nervous activity, and, as possible counterregulating system...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF01907058
更新日期：1987-03-01 00:00:00
abstract：:We have characterized the aerobic blood-perfused isolated heart model evaluating the hemodynamics and metabolism of both the blood donor animal and the isolated organ. Anaesthesia of the blood donor with sodium pentobarbital (30 mg/kg) increases arterial concentration of non esterified fatty acids (NEFA) from 80 +/- 6...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/s003950050145
更新日期：1999-06-01 00:00:00
abstract：OBJECTIVE:The clinical usefulness of class III antiarrhythmic drugs for the treatment of tachyarrhythmias is limited by their potential proarrhythmic effects, mainly torsades-depointes (TdP). The goal of this experimental study was to develop an isolated whole-heart model exhibiting typical characteristics of class III...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/s003950050073
更新日期：1998-04-01 00:00:00
abstract：:In anaesthetized, artificially respirated mongrel dogs, the intravenous infusion of oxprenolol (0.3 mg and 1 mg/kg over 10 minutes) led to an increase in heart rate and cardiac output. Blood pressure in the pulmonary capillaries and right atrium, and vascular resistance were reduced. The pressure in the aorta was tran...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF01906388
更新日期：1975-09-01 00:00:00
abstract：:Endothelin-1 elicited a concentration-dependent positive inotropic effect on the isolated rabbit, guinea pig, and rat, but not on the dog ventricular myocardium. The duration of isometric contractions was prolonged by endothelin-1 in a concentration-dependent manner, mainly by prolongation of relaxation time. Phorbol ...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：
更新日期：1991-01-01 00:00:00
abstract：OBJECTIVES:The impact of acute collagen disruption by the disulfide donor, 5,5'-dithio-2-nitrobenzoic acid (DTNB) on ventricular properties was tested in rat hearts. METHODS:Collagen was degraded acutely in 13 isolated, isovolumically contracting rat hearts by perfusion with 1 mM DTNB added to Krebs-Henseleit solution...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF00788632
更新日期：1997-06-01 00:00:00
abstract：:The characterization of various ion transport systems has led to a better understanding of the effects, which seem to take part in the impairment of ischemic and reperfused cardiac tissue. This review discusses the role of the Na+/H+ exchange system in the pathophysiology of ischemia and reperfusion and the beneficial...
journal_title：Basic research in cardiology
pub_type： 杂志文章,评审
doi：10.1007/BF00795411
更新日期：1993-09-01 00:00:00
abstract：:The pathogenesis and progression of hypertensive heart disease are unclear; however, both involve a genetic predisposition and environmental influences. To test the role of exogenous factors, we examined the hearts of spontaneously hypertensive rats (SHR) exposed to noise stress and ethanol intake. Twenty-two SHR and ...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF00794951
更新日期：1994-11-01 00:00:00
abstract：:Exercise is an efficient strategy for myocardial protection against ischemia-reperfusion (IR) injury. Although endothelial nitric oxide synthase (eNOS) is phosphorylated and activated during exercise, its role in exercise-induced cardioprotection remains unknown. This study investigated whether modulation of eNOS acti...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/s00395-013-0389-2
更新日期：2013-11-01 00:00:00
abstract：:Hypertension and resulting left ventricular hypertrophy was induced in young male Wistar rats (60 to 70 days old) by narrowing of one renal artery (Goldblatt II). 8 and 24 weeks after operation, myocardial oxygen consumption was measured on a modified in situ heart-lung preparation with nearly isovolumetric left ventr...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF01908041
更新日期：1982-05-01 00:00:00
abstract：:Long QT syndrome is a potentially life-threatening disease characterized by delayed repolarization of cardiomyocytes, QT interval prolongation in the electrocardiogram, and a high risk for sudden cardiac death caused by ventricular arrhythmia. The genetic type 3 of this syndrome (LQT3) is caused by gain-of-function mu...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/s00395-016-0530-0
更新日期：2016-03-01 00:00:00
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journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF01908204
更新日期：1989-09-01 00:00:00
abstract：:An adult rat heart myocyte preparation was used to study the uptake and metabolism of the 1-14C-labelled fatty acids decanoate, laurate, myristate, and palmitate. Metabolism was saturable, but free fatty acid accumulation was not. Use of metabolic inhibitors indicated that metabolism and not uptake was rate limiting a...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：
更新日期：1985-01-01 00:00:00
abstract：:It is generally believed that the increased contractility and tachycardia of the hyperthyroid heart are a result of thyroid hormone-induced alterations of the mechanical and electrical properties of the heart, respectively. We compared the contractility (dP/dtmax) and the spontaneous beating rate of hyperthyroid and e...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF01906958
更新日期：1988-11-01 00:00:00
abstract：OBJECTIVES:Single electrical field shocks are able to induce ventricular fibrillation (VF) if applied during the vulnerable period. During this period, a shock can either prolong the action potential duration or induce a new action potential. Whether the occurrence of different shock responses contributes to the induct...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF00803755
更新日期：1997-02-01 00:00:00
abstract：:Thyroid hormone (TH) is critical for tissue differentiation at early stages of development, induces physiological hypertrophy and regulates the expression of important contractile proteins such as myosin heavy chain (MHC) isoform and calcium cycling proteins. Furthermore, TH seems to control the response to stress by ...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/s00395-008-0697-0
更新日期：2008-07-01 00:00:00
abstract：:There is no doubt that major advances have been made in cardiac electrophysiology using single heart cells. Accurate analysis of rapid inward sodium current and the second inward current carried by calcium has resulted in a major re-examination of the steps involved in excitation-contraction coupling in the heart. Fut...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/978-3-662-11041-6_17
更新日期：1985-01-01 00:00:00
abstract：:The influence of perfusion pressure (5-8 kPa) on coronary flow and myocardial Ca2+-washout during Ca2+-free perfusion, and on the occurrence of calcium paradox after subsequent Ca2+-reperfusion, was studied in isolated, stimulated, rat heart ventricles. During the Ca2+-free perfusion period (3 min) the following chang...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF01907580
更新日期：1980-03-01 00:00:00
abstract：:Increase of Ca2+ concentration to 7.5 mM in the presence of 10 mM or 20 mM caffeine shifted length-tension relationships of 5 rat trabecular muscles and 5 cat papillary muscles to higher tensions. At 1max diastolic tension was enhanced from (formula: see text), for all measured points of stress-strain relationships af...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF01906525
更新日期：1978-09-01 00:00:00
abstract：:Myocytes isolated from guinea pig ventricles were voltage-clamped using patch pipettes in the whole-cell configuration. For proper voltage control fast Na+ current was blocked by TTX or inactivated by an appropriate prepulse. Zero-load cell shortening was monitored by a photoelectric device. The mechanical response to...
journal_title：Basic research in cardiology
pub_type： 杂志文章
doi：10.1007/BF01907923
更新日期：1989-03-01 00:00:00
