Pathophysiological Mechanisms of Sino-Atrial Dysfunction and Ventricular Conduction Disease Associated with SCN5A Deficiency: Insights from Mouse Models.

Abstract:

:Genetically modified mice provide a number of models for studying cardiac channelopathies related to cardiac Na(+) channel (SCN5A) abnormalities. We review key pathophysiological features in these murine models that may underlie clinical features observed in sinus node dysfunction and progressive cardiac conduction disease, thereby providing insights into their pathophysiological mechanisms. We describe loss of Na(+) channel function and fibrotic changes associated with both loss and gain-of-function Na(+) channel mutations. Recent reports further relate the progressive fibrotic changes to upregulation of TGF-β1 production and the transcription factors, Atf3, a stress-inducible gene, and Egr1, to the presence of heterozygous Scn5a gene deletion. Both changes are thus directly implicated in the clinically observed disruptions in sino-atrial node pacemaker function, and sino-atrial and ventricular conduction, and their progression with age. Murine systems with genetic modifications in Scn5a thus prove a useful tool to address questions concerning roles of genetic and environmental modifiers on human SCN5A disease phenotypes.

journal_name

Front Physiol

journal_title

Frontiers in physiology

authors

Huang CL,Lei L,Matthews GD,Zhang Y,Lei M

doi

10.3389/fphys.2012.00234

subject

Has Abstract

pub_date

2012-07-06 00:00:00

pages

234

issn

1664-042X

journal_volume

3

pub_type

杂志文章
  • Nanotherapeutics for Treatment of Pulmonary Arterial Hypertension.

    abstract::Pulmonary arterial hypertension (PAH) is a devastating and fatal chronic lung disease. While current pharmacotherapies have improved patient quality of life, PAH drugs suffer from limitations in the form of short-term pharmacokinetics, instability, and poor organ specificity. Traditionally, nanotechnology-based delive...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章,评审

    doi:10.3389/fphys.2018.00890

    authors: Segura-Ibarra V,Wu S,Hassan N,Moran-Guerrero JA,Ferrari M,Guha A,Karmouty-Quintana H,Blanco E

    更新日期:2018-07-13 00:00:00

  • Different Types of Physical Activity and Metabolic Control in People With Type 1 Diabetes Mellitus.

    abstract:Background:The aim of presented cross-sectional study was to determine the association of different types of physical activity (PA) with metabolic control in people with type 1 diabetes. Materials and Methods:A total of 109 adult subjects with type 1 diabetes were asked to complete the non-exercise activity thermogene...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2019.01210

    authors: Štotl I,Kambič T,Hadžić V,Zdolšek A

    更新日期:2019-09-24 00:00:00

  • Proton-Translocating Nicotinamide Nucleotide Transhydrogenase: A Structural Perspective.

    abstract::Nicotinamide nucleotide transhydrogenase (TH) is an enzyme complex in animal mitochondria and bacteria that utilizes the electrochemical proton gradient across membranes to drive the production of NADPH. The enzyme plays an important role in maintaining the redox balance of cells with implications in aging and a numbe...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章,评审

    doi:10.3389/fphys.2017.01089

    authors: Zhang Q,Padayatti PS,Leung JH

    更新日期:2017-12-19 00:00:00

  • Resting state MEG oscillations show long-range temporal correlations of phase synchrony that break down during finger movement.

    abstract::The capacity of the human brain to interpret and respond to multiple temporal scales in its surroundings suggests that its internal interactions must also be able to operate over a broad temporal range. In this paper, we utilize a recently introduced method for characterizing the rate of change of the phase difference...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2015.00183

    authors: Botcharova M,Berthouze L,Brookes MJ,Barnes GR,Farmer SF

    更新日期:2015-06-17 00:00:00

  • Identification of Halloween Genes and RNA Interference-Mediated Functional Characterization of a Halloween Gene shadow in Plutella xylostella.

    abstract::Ecdysteroids play an essential role in controlling insect development and reproduction. Their pathway is regulated by a group of enzymes called Halloween gene proteins. The relationship between the Halloween genes and ecdysteroid synthesis has yet to be clearly understood in diamondback moth, Plutella xylostella (L.),...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2019.01120

    authors: Peng L,Wang L,Zou MM,Vasseur L,Chu LN,Qin YD,Zhai YL,You MS

    更新日期:2019-08-28 00:00:00

  • Glutamate Transport and Preterm Brain Injury.

    abstract::Preterm birth complications are the leading cause of child death worldwide and a top global health priority. Among the survivors, the risk of life-long disabilities is high, including cerebral palsy and impairment of movement, cognition, and behavior. Understanding the molecular mechanisms of preterm brain injuries is...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章,评审

    doi:10.3389/fphys.2019.00417

    authors: Pregnolato S,Chakkarapani E,Isles AR,Luyt K

    更新日期:2019-04-24 00:00:00

  • Clinical Applications of Iso-Inertial, Eccentric-Overload (YoYo™) Resistance Exercise.

    abstract::In the quest for a viable non-gravity dependent method to "lift weights" in space, our laboratory introduced iso-inertial resistance (YoYo™) exercise using spinning flywheel(s), more than 25 years ago. After being thoroughly tested in individuals subjected to various established spaceflight analogs, a multi-mode YoYo™...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章,评审

    doi:10.3389/fphys.2017.00241

    authors: Tesch PA,Fernandez-Gonzalo R,Lundberg TR

    更新日期:2017-04-27 00:00:00

  • Zebrafish Circadian Clock Entrainment and the Importance of Broad Spectral Light Sensitivity.

    abstract::One of the key defining features of an endogenous circadian clock is that it can be entrained or set to local time. Though a number of cues can perform this role, light is the predominant environmental signal that acts to entrain circadian pacemakers in most species. For the past 20 years, a great deal of work has bee...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2020.01002

    authors: Steindal IAF,Whitmore D

    更新日期:2020-08-14 00:00:00

  • Vesicle-Mediated Control of Cell Function: The Role of Extracellular Matrix and Microenvironment.

    abstract::Extracellular vesicles (EVs) - including exosomes, microvesicles and apoptotic bodies - have received much scientific attention last decade as mediators of a newly discovered cell-to-cell communication system, acting at short and long distances. EVs carry biologically active molecules, thus providing signals that infl...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章,评审

    doi:10.3389/fphys.2018.00651

    authors: Rackov G,Garcia-Romero N,Esteban-Rubio S,Carrión-Navarro J,Belda-Iniesta C,Ayuso-Sacido A

    更新日期:2018-06-05 00:00:00

  • Effects of Topical Icing on Inflammation, Angiogenesis, Revascularization, and Myofiber Regeneration in Skeletal Muscle Following Contusion Injury.

    abstract::Contusion injuries in skeletal muscle commonly occur in contact sport and vehicular and industrial workplace accidents. Icing has traditionally been used to treat such injuries under the premise that it alleviates pain, reduces tissue metabolism, and modifies vascular responses to decrease swelling. Previous research ...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2017.00093

    authors: Singh DP,Barani Lonbani Z,Woodruff MA,Parker TJ,Steck R,Peake JM

    更新日期:2017-03-07 00:00:00

  • Heart Rate Is a Better Predictor of Cardiorespiratory Fitness Than Heart Rate Variability in Overweight/Obese Children: The ActiveBrains Project.

    abstract::Cardiac autonomic function can be quantified through mean heart rate (HR) or heart rate variability (HRV). Numerous studies have supported the utility of different HRV parameters as indicators of cardiorespiratory fitness (CRF). However, HR has recently shown to be a stronger predictor of CRF than HRV in healthy young...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2019.00510

    authors: Plaza-Florido A,Migueles JH,Mora-Gonzalez J,Molina-Garcia P,Rodriguez-Ayllon M,Cadenas-Sanchez C,Esteban-Cornejo I,Solis-Urra P,de Teresa C,Gutiérrez Á,Michels N,Sacha J,Ortega FB

    更新日期:2019-05-07 00:00:00

  • Regulation of arterial pressure by the paraventricular nucleus in conscious rats: interactions among glutamate, GABA, and nitric oxide.

    abstract::The paraventricular nucleus (PVN) of the hypothalamus is an important site for autonomic and neuroendocrine regulation. Experiments in anesthetized animals and in vitro indicate an interaction among gamma-aminobutyric acid (GABA), nitric oxide (NO), and glutamate in the PVN. The cardiovascular role of the PVN and inte...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2012.00490

    authors: Martins-Pinge MC,Mueller PJ,Foley CM,Heesch CM,Hasser EM

    更新日期:2013-01-09 00:00:00

  • Effect of phenylephrine vs. ephedrine on frontal lobe oxygenation during caesarean section with spinal anesthesia: an open label randomized controlled trial.

    abstract:BACKGROUND:During caesarean section spinal anesthesia may provoke maternal hypotension that we prevent by administration of phenylephrine and/or ephedrine. Phenylephrine is however reported to reduce the near infrared spectroscopy-determined frontal lobe oxygenation (ScO2) but whether that is the case for patients expo...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2014.00081

    authors: Foss VT,Christensen R,Rokamp KZ,Nissen P,Secher NH,Nielsen HB

    更新日期:2014-03-03 00:00:00

  • Regulators of Collagen Fibrillogenesis during Molar Development in the Mouse.

    abstract::Development of mammalian teeth and surrounding tissues includes time-space changes in the extracellular matrix composition and organization. This requires complex control mechanisms to regulate its synthesis and remodeling. Fibril-associated collagens with interrupted triple helices (FACITs) and a group of small leuci...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2017.00554

    authors: Zvackova I,Matalova E,Lesot H

    更新日期:2017-08-02 00:00:00

  • Crayfish Self-Administer Amphetamine in a Spatially Contingent Task.

    abstract::Natural reward is an essential element of any organism's ability to adapt to environmental variation. Its underlying circuits and mechanisms guide the learning process as they help associate an event, or cue, with the perception of an outcome's value. More generally, natural reward serves as the fundamental generator ...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2018.00433

    authors: Datta U,van Staaden M,Huber R

    更新日期:2018-05-14 00:00:00

  • Distinctive Steady-State Heart Rate and Blood Pressure Responses to Passive Robotic Leg Exercise and Functional Electrical Stimulation during Head-Up Tilt.

    abstract::Introduction: Tilt tables enable early mobilization of patients by providing verticalization. But there is a high risk of orthostatic hypotension provoked by verticalization, especially after neurological diseases such as spinal cord injury. Robot-assisted tilt tables might be an alternative as they add passive roboti...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2016.00612

    authors: Sarabadani Tafreshi A,Riener R,Klamroth-Marganska V

    更新日期:2016-12-09 00:00:00

  • Apelin Inhibits Angiotensin II-Induced Atrial Fibrosis and Atrial Fibrillation via TGF-β1/Smad2/α-SMA Pathway.

    abstract:Background:Angiotensin II (Ang II) could promote the development of atrial fibrosis in atrial fibrillation (AF). Apelin can inhibit the occurrence of myocardial fibrosis. However, the effect of apelin on Ang II-induced atrial fibrosis and subsequent AF still remains unknown. Objective:In the present study, we examined...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2020.583570

    authors: Lv W,Zhang L,Cheng X,Wang H,Qin W,Zhou X,Tang B

    更新日期:2020-11-19 00:00:00

  • Personalized Cardiovascular Disease Prediction and Treatment-A Review of Existing Strategies and Novel Systems Medicine Tools.

    abstract::Cardiovascular disease (CVD) continues to constitute the leading cause of death globally. CVD risk stratification is an essential tool to sort through heterogeneous populations and identify individuals at risk of developing CVD. However, applications of current risk scores have recently been shown to result in conside...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章,评审

    doi:10.3389/fphys.2016.00002

    authors: Björnson E,Borén J,Mardinoglu A

    更新日期:2016-01-26 00:00:00

  • Chloride Influx of Anion Exchanger 2 Was Modulated by Calcium-Dependent Spinophilin in Submandibular Glands.

    abstract::Secretory glands including salivary glands by many hormonal inputs produce and secrete biological fluids determined by variety of ion transporters. Spinophilin is a multifunctional scaffolding protein, which involved in receptor signaling and regulation of anion exchangers AE2 activity. We found that spinophilin expre...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2018.00889

    authors: Lee D,Lee SA,Shin DM,Hong JH

    更新日期:2018-07-19 00:00:00

  • Redox Aspects of Chaperones in Cardiac Function.

    abstract::Molecular chaperones are stress proteins that allow the correct folding or unfolding as well as the assembly or disassembly of macromolecular cellular components. Changes in expression and post-translational modifications of chaperones have been linked to a number of age- and stress-related diseases including cancer, ...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章,评审

    doi:10.3389/fphys.2018.00216

    authors: Penna C,Sorge M,Femminò S,Pagliaro P,Brancaccio M

    更新日期:2018-03-16 00:00:00

  • The Effect of Physical Activity on Cognitive Performance in an Italian Elementary School: Insights From a Pilot Study Using Structural Equation Modeling.

    abstract::When compared to the previous generations, younger generations have become sedentary on a global level. Physical activity positively contributes to human growth and development, causing, indeed, both physiological and psychological benefits. The aim of the current study was examining the relationship between physical ...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2019.00202

    authors: Padulo J,Bragazzi NL,De Giorgio A,Grgantov Z,Prato S,Ardigò LP

    更新日期:2019-03-05 00:00:00

  • Increased Reactive Oxygen Species and Cell Cycle Defects Contribute to Anemia in the RASA3 Mutant Mouse Model scat.

    abstract::RASA3 is a Ras GTPase activating protein that plays a critical role in blood formation. The autosomal recessive mouse model scat (severe combined anemia and thrombocytopenia) carries a missense mutation in Rasa3. Homozygotes present with a phenotype characteristic of bone marrow failure that is accompanied by alternat...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2018.00689

    authors: Hartman ES,Brindley EC,Papoin J,Ciciotte SL,Zhao Y,Peters LL,Blanc L

    更新日期:2018-06-05 00:00:00

  • Identification of Biologically Essential Nodes via Determinative Power in Logical Models of Cellular Processes.

    abstract::A variety of biological networks can be modeled as logical or Boolean networks. However, a simplification of the reality to binary states of the nodes does not ease the difficulty of analyzing the dynamics of large, complex networks, such as signal transduction networks, due to the exponential dependence of the state ...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2018.01185

    authors: Pentzien T,Puniya BL,Helikar T,Matache MT

    更新日期:2018-08-31 00:00:00

  • The MEK-Inhibitor Selumetinib Attenuates Tumor Growth and Reduces IL-6 Expression but Does Not Protect against Muscle Wasting in Lewis Lung Cancer Cachexia.

    abstract::Cachexia, or wasting of skeletal muscle and fat, afflicts many patients with chronic diseases including cancer, organ failure, and AIDS. Muscle wasting reduces quality of life and decreases response to therapy. Cachexia is caused partly by elevated inflammatory cytokines, including interleukin-6 (IL-6). Others and we ...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2016.00682

    authors: Au ED,Desai AP,Koniaris LG,Zimmers TA

    更新日期:2017-01-18 00:00:00

  • Are mouse models of asthma appropriate for investigating the pathogenesis of airway hyper-responsiveness?

    abstract::Whether mouse models of chronic asthma can be used to investigate the relationship between airway inflammation/remodeling and airway hyper-responsiveness (AHR) is a vexed question. It raises issues about the extent to which such models replicate key features of the human disease. Here, we review some of the characteri...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2012.00312

    authors: Kumar RK,Foster PS

    更新日期:2012-07-31 00:00:00

  • Sedentary Behavior Is Only Marginally Associated with Physical Function in Adults Aged 40-75 Years-the Maastricht Study.

    abstract::Background: In an aging population, regular physical activity (PA) and exercise have been recognized as important factors in maintaining physical function and thereby preventing loss of independence and disability. However, (older) adults spent the majority of their day sedentary and therefore insight into the consequ...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2017.00242

    authors: van der Velde JHPM,Savelberg HHCM,van der Berg JD,Sep SJS,van der Kallen CJH,Dagnelie PC,Schram MT,Henry RMA,Reijven PLM,van Geel TACM,Stehouwer CDA,Koster A,Schaper NC

    更新日期:2017-04-25 00:00:00

  • Glutamate Cysteine Ligase-Modulatory Subunit Knockout Mouse Shows Normal Insulin Sensitivity but Reduced Liver Glycogen Storage.

    abstract::Glutathione (GSH) deficits have been observed in several mental or degenerative illness, and so has the metabolic syndrome. The impact of a decreased glucose metabolism on the GSH system is well-known, but the effect of decreased GSH levels on the energy metabolism is unclear. The aim of the present study was to inves...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2016.00142

    authors: Lavoie S,Steullet P,Kulak A,Preitner F,Do KQ,Magistretti PJ

    更新日期:2016-04-21 00:00:00

  • The blue-collar brain.

    abstract::Much effort has gone into elucidating control of the body by the brain, less so the role of the body in controlling the brain. This essay develops the idea that the brain does a great deal of work in the service of behavior that is controlled by the body, a blue-collar role compared to the white-collar control exercis...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2012.00207

    authors: Van Orden G,Hollis G,Wallot S

    更新日期:2012-06-18 00:00:00

  • Propofol Alleviates DNA Damage Induced by Oxygen Glucose Deprivation and Reperfusion via FoxO1 Nuclear Translocation in H9c2 Cells.

    abstract::Ischemia/reperfusion (I/R) injury induces irreversible oxidative stress damage to the cardiac myocytes. Many studies have revealed that propofol alleviates the important organelle-mediated injury from oxidative stress in vitro. However, it remains unclear whether propofol prevents I/R-induced DNA damage in cardiomyocy...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2019.00223

    authors: Zhou D,Zhuang J,Wang Y,Zhao D,Zhao L,Zhu S,Pu J,Yin M,Zhang H,Wang Z,Hong J

    更新日期:2019-03-15 00:00:00

  • The Effect of the Stretch-Shortening Cycle in the Force-Velocity Relationship and Its Association With Physical Function in Older Adults With COPD.

    abstract::This study aimed to evaluate the effect of the stretch-shortening cycle (SSC) on different portions of the force-velocity (F-V) relationship in older adults with and without chronic obstructive pulmonary disease (COPD), and to assess its association with physical function. The participants were 26 older adults with CO...

    journal_title:Frontiers in physiology

    pub_type: 杂志文章

    doi:10.3389/fphys.2019.00316

    authors: Navarro-Cruz R,Alcazar J,Rodriguez-Lopez C,Losa-Reyna J,Alfaro-Acha A,Ara I,García-García FJ,Alegre LM

    更新日期:2019-03-26 00:00:00