The role of branched fibres in the pathogenesis of Duchenne muscular dystrophy.

Abstract:

:Branched fibres are a well-documented phenomenon of regenerating skeletal muscle. They are found in the muscles of boys with Duchenne muscular dystrophy (DMD), a severe condition of progressive muscle wasting caused by an absence of the sarcolemmal protein dystrophin, and in the muscles of the mdx mouse, an animal model of DMD. However, only a handful of studies have investigated how the physiological properties of these morphologically deformed fibres differ from those of normal fibres. These studies have found an association between the extent of fibre branching in mdx muscles and the susceptibility of these muscles to damage from eccentric contractions. They have also found that branched mdx muscle fibres cannot sustain maximal contractions in buffered Ca(2+) solutions, that branch points are sites of increased mechanical stress and that myofibrillar structure is greatly disturbed at branch points. These findings have important implications for understanding the function of dystrophin. It is commonly thought that the role of dystrophin is mechanical stabilization of the sarcolemma, as numerous studies have shown that eccentric contractions damage mdx muscle more than normal muscle. However, the finding that branched mdx fibres are mechanically weakened raises the question, is it the lack of dystrophin or is it the fibre branching that leads to the vulnerability of mdx muscle to contractile damage? The importance of this question to our understanding of the function of dystrophin warrants further research into the physiological properties of branched fibres and how they differ from morphologically normal fibres.

journal_name

Exp Physiol

journal_title

Experimental physiology

authors

Chan S,Head SI

doi

10.1113/expphysiol.2010.056713

subject

Has Abstract

pub_date

2011-06-01 00:00:00

pages

564-71

issue

6

eissn

0958-0670

issn

1469-445X

pii

expphysiol.2010.056713

journal_volume

96

pub_type

杂志文章,评审
  • The effect of chronic sodium depletion on renal function in conscious rats.

    abstract::In order to investigate the effect of chronic sodium depletion on renal proximal tubular reabsorption, studies were performed in conscious, unrestrained Brattleboro rats. Since these animals lack circulating vasopressin, fractional water reabsorption in the distal nephron can be assumed to be constant and changes in u...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/expphysiol.1994.sp003749

    authors: Shirley DG,Skinner J

    更新日期:1994-03-01 00:00:00

  • Modulation of voltage-dependent calcium current in Helix aspersa buccal neurones by serotonin and protein kinase C activators.

    abstract::In Helix aspersa, activation of the cerebral giant serotonin neurones (GSNs) evokes a biphasic, excitatory synaptic response in the M neurones of the buccal ganglia. Local application of serotonin to the current-clamped M neurones also evokes fast and slow depolarizing responses. The slow response is thought to be dep...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/expphysiol.1992.sp003656

    authors: Hill-Venning C,Cottrell GA

    更新日期:1992-11-01 00:00:00

  • Influence of muscle fibre type and fitness on the oxygen uptake/power output slope during incremental exercise in humans.

    abstract::We recently reported that a higher percentage of type I fibres in vastus lateralis and a greater peak oxygen uptake (O2) were associated with a greater initial rise in O2 (O2 /W, where W is work rate) following the onset of heavy constant power output exercise (above the lactate threshold, LT). It was unclear if these...

    journal_title:Experimental physiology

    pub_type: 临床试验,杂志文章

    doi:

    authors: Barstow TJ,Jones AM,Nguyen PH,Casaburi R

    更新日期:2000-01-01 00:00:00

  • Endothelial hypoxic preconditioning in rat hypoxic isolated aortic segments.

    abstract::Our aim was to analyse endothelial hypoxic preconditioning after hypoxia-reperfusion (HR). Endothelial functionality was analysed through the vasorelaxation responses to acetylcholine (Ach) and the level of serine1177 phosphorylated endothelial nitric oxide synthase (eNOS) (ser1177-eNOS) measured by Western blot in in...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/expphysiol.2005.030163

    authors: Carrasco-Martín C,Alonso-Orgaz S,De la Pinta JC,Marques M,Macaya C,Barrientos A,González MM,García-Méndez A,Mateos-Cáceres PJ,Porres JC,Rico LA,López-Farré AJ

    更新日期:2005-07-01 00:00:00

  • Daily patterns of secretion of neurohypophysial hormones in man: effect of age.

    abstract::The neurohypophysial hormone vasopressin contributes to control of urine output and, while urine flow shows a clear daily rhythm, there has been debate as to whether this is true of neurohypophysial hormones. A study was performed on fifteen adult males, with a mean age of 25 years, over a 24 h period, nine blood samp...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/expphysiol.1998.sp004124

    authors: Forsling ML,Montgomery H,Halpin D,Windle RJ,Treacher DF

    更新日期:1998-05-01 00:00:00

  • A faster twitch contraction of soleus in the spontaneously hypertensive rat is partly due to changed fibre type composition.

    abstract::Contractile properties and fibre type composition of slow-twitch soleus muscles from spontaneously hypertensive rats (SHR) were compared with those from a control normotensive strain (Wistar-Kyoto, WKY). Male rats aged around 100 days were used, and muscle tension was recorded isometrically in vitro. Tetanic tension w...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/expphysiol.1994.sp003772

    authors: Lewis DM,Levi AJ,Brooksby P,Jones JV

    更新日期:1994-05-01 00:00:00

  • Whole cell recordings from respiratory neurones in an arterially perfused in situ neonatal rat preparation.

    abstract::For synaptic and cellular analyses of the mammalian respiratory network, intracellular recording and good pharmacological access to respiratory neurones is required. Using an existing arterially perfused in situ preparation of neonatal rat, we report on a method allowing stable intracellular recordings of ventrolatera...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/eph8802639

    authors: Dutschmann M,Paton JF

    更新日期:2003-11-01 00:00:00

  • Long non-coding RNA PRRT3-AS1 silencing inhibits prostate cancer cell proliferation and promotes apoptosis and autophagy.

    abstract:NEW FINDINGS:What is the central question of this study? What is the role of lncRNA PRRT3-AS1 in the regulation of peroxisome proliferator-activated receptor γ (PPARγ) gene-mediated mechanistic target of rapamycin (mTOR) signalling pathway in proliferation, apoptosis and autophagy of prostate cancer cells? What is the ...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/EP088011

    authors: Fan L,Li H,Wang W

    更新日期:2020-05-01 00:00:00

  • Vascular responses to hypoxia are not impaired in obstructive sleep apnoea patients free of overt cardiovascular disease.

    abstract:NEW FINDINGS:What is the central question of this study? Does treatment of obstructive sleep apnoea (OSA) with nocturnal oxygen or continuous positive airway pressure (CPAP) improve hypoxic vascular responses, which are reportedly impaired in OSA? What is the main finding and its importance? Cerebrovascular and cardiov...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/EP086845

    authors: Beaudin AE,Hanly PJ,Raneri JK,Sajobi TT,Anderson TJ,Poulin MJ

    更新日期:2019-04-01 00:00:00

  • REM sleep-like episodes of motoneuronal depression and respiratory rate increase are triggered by pontine carbachol microinjections in in situ perfused rat brainstem preparation.

    abstract::Hypoglossal nerve activity (HNA) controls the position and movements of the tongue. In persons with compromised upper airway anatomy, sleep-related hypotonia of the tongue and other pharyngeal muscles causes increased upper airway resistance, or total upper airway obstructions, thus disrupting both sleep and breathing...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/expphysiol.2010.056242

    authors: Brandes IF,Stettner GM,Mörschel M,Kubin L,Dutschmann M

    更新日期:2011-05-01 00:00:00

  • The increase in body weight induced by lack of methyl CpG binding protein-2 is associated with altered leptin signalling in the hypothalamus.

    abstract::Methyl CpG binding protein-2 (MECP2) is a chromatin-remodelling factor with a dual role in gene expression. Evidence from patients carrying MECP2 mutations and from transgenic mouse models demonstrates that this protein is involved in the control of body weight. However, the mechanism for this has not been fully eluci...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/expphysiol.2014.079798

    authors: Torres-Andrade R,Moldenhauer R,Gutierrez-Bertín N,Soto-Covasich J,Mancilla-Medina C,Ehrenfeld C,Kerr B

    更新日期:2014-09-01 00:00:00

  • Programmed cell death in bovine mammary tissue during lactation and involution.

    abstract::Cessation of milk removal causes mammary tissue involution, which in rodents is characterized by extensive tissue degeneration and loss of the majority of luminal epithelial cells by apoptosis. In contrast, bovine mammary tissue shows little histological evidence of tissue remodelling between lactations. In this study...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/expphysiol.1997.sp004075

    authors: Wilde CJ,Addey CV,Li P,Fernig DG

    更新日期:1997-09-01 00:00:00

  • Consecutive transcranial magnetic stimulation twitches reduce flow limitation during sleep in apnoeic patients.

    abstract:NEW FINDINGS:What is the central question of this study? A transcranial magnetic stimulation (TMS)-induced twitch applied on isolated single breaths over the motor cortex somatotopic representation of the tongue briefly recruits submental muscles and improves airflow dynamics of flow-limited respiratory cycles without ...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/expphysiol.2013.073072

    authors: Melo-Silva CA,Gakwaya S,Rousseau E,Sériès F

    更新日期:2013-09-01 00:00:00

  • Acute phase response to exertional heat stroke in mice.

    abstract:NEW FINDINGS:What is the central question of this study? Exertional heat stroke is accompanied by a marked inflammatory response. In this study, we explored the time course of acute phase proteins during recovery from severe heat stress in mice and the potential role of skeletal muscles as their source. What is the mai...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/EP088501

    authors: Iwaniec J,Robinson GP,Garcia CK,Murray KO,de Carvalho L,Clanton TL,Laitano O

    更新日期:2021-01-01 00:00:00

  • 5-Aminoimidazole-4-carboxamide ribonucleotide prevents fat gain following the cessation of voluntary physical activity.

    abstract:NEW FINDINGS:What is the central question of this study? We investigated whether 5-aminoimidazole-4-carboxamide-1-beta-D-ribofuranoside (AICAR) could prevent acute increases in body fat and changes in omental and subcutaneous adipose tissue following the sudden transition from physical activity to physical inactivity. ...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/EP086335

    authors: Ruegsegger GN,Sevage JA,Childs TE,Grigsby KB,Booth FW

    更新日期:2017-11-01 00:00:00

  • Metabolic plasticity mediates differential responses to spring and autumn migrations: Evidence from gene expression patterns in migratory buntings.

    abstract:NEW FINDINGS:What is the central question of this study? What are the molecular underpinnings of seasonal metabolic plasticity during spring and autumn migrations in songbirds? What is the main finding and its importance? We report differences in mRNA levels of genes involved in the regulation of glucose and fat metabo...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/EP087974

    authors: Sharma A,Kumar V

    更新日期:2019-12-01 00:00:00

  • Cardiomyocyte death and the ageing and failing heart.

    abstract::Mammalian cardiomyocytes have limited regenerative capacity, such that cell death can result in a net loss of viable contractile elements and a decrease in cardiac functional reserve, both during normal ageing and after insults to the myocardium leading to heart failure. At least four types of cell death have been des...

    journal_title:Experimental physiology

    pub_type: 杂志文章,评审

    doi:10.1113/eph8802549

    authors: Goldspink DF,Burniston JG,Tan LB

    更新日期:2003-05-01 00:00:00

  • Respiratory disturbances in a mouse model of Parkinson's disease.

    abstract:NEW FINDINGS:What is the central question of this study? Clinical reports have described and suggested central and peripheral respiratory abnormalities in Parkinson's disease (PD) patients; however, these reports have never addressed the occurrence of these abnormalities in an animal model. What is the main finding and...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/EP087507

    authors: Oliveira LM,Oliveira MA,Moriya HT,Moreira TS,Takakura AC

    更新日期:2019-05-01 00:00:00

  • Nerve growth factor-dependent hyperexcitability of capsaicin-sensitive bladder afferent neurones in mice with spinal cord injury.

    abstract:NEW FINDINGS:What is the central question of this study? Nerve growth factor (NGF) is reportedly a mediator inducing urinary bladder dysfunction. Is NGF directly involved in hyperexcitability of capsaicin-sensitive C-fibre bladder afferent pathways after spinal cord injury (SCI)? What is the main finding and its import...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/EP086951

    authors: Shimizu T,Majima T,Suzuki T,Shimizu N,Wada N,Kadekawa K,Takai S,Takaoka E,Kwon J,Kanai AJ,de Groat WC,Tyagi P,Saito M,Yoshimura N

    更新日期:2018-06-01 00:00:00

  • Double-sigmoid model for fitting fatigue profiles in mouse fast- and slow-twitch muscle.

    abstract::We present a curve-fitting approach that permits quantitative comparisons of fatigue profiles obtained with different stimulation protocols in isolated slow-twitch soleus and fast-twitch extensor digitorum longus (EDL) muscles of mice. Profiles from our usual stimulation protocol (125 Hz for 500 ms, evoked once every ...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/expphysiol.2007.041285

    authors: Cairns SP,Robinson DM,Loiselle DS

    更新日期:2008-07-01 00:00:00

  • Permeability characteristics of monovalent cations in atrial myocytes of the rat heart.

    abstract::We investigated the permeability of Cs+ and Na+ through various ion channels in rat atrial myocytes using the whole-cell voltage-clamp technique. With isotonic CsCl (140 mM) on both sides of the membrane and nominally [Ca2+]o-free conditions, depolarising clamp pulses induced an increase of outward currents which show...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:

    authors: Youm JB,Ho WK,Earm YE

    更新日期:2000-03-01 00:00:00

  • Human hypertension, sympathetic activity and the selfish brain.

    abstract:NEW FINDINGS:What is the topic of this review? This review article revisits an historical hypothesis that cerebral hypoperfusion, caused by elevated cerebral vascular resistances, causes the onset of high sympathetic nerve activity and hypertension in humans. What advances does it highlight? The review article highligh...

    journal_title:Experimental physiology

    pub_type: 杂志文章,评审

    doi:10.1113/EP085775

    authors: Hart EC

    更新日期:2016-12-01 00:00:00

  • Defibrillation of the heart: insights into mechanisms from modelling studies.

    abstract::Despite its critical role in restoring cardiac rhythm and thus in saving human life, cardiac defibrillation remains poorly understood. Further mechanistic inquiry is hampered by the inability of presently available experimental techniques to resolve, with sufficient accuracy, electrical behaviour confined to the depth...

    journal_title:Experimental physiology

    pub_type: 杂志文章,评审

    doi:10.1113/expphysiol.2005.030973

    authors: Trayanova N

    更新日期:2006-03-01 00:00:00

  • Spontaneously hypertensive rats have more orexin neurons in the hypothalamus and enhanced orexinergic input and orexin 2 receptor-associated nitric oxide signalling in the rostral ventrolateral medulla.

    abstract:NEW FINDINGS:What is the central question of this study? Our previous study demonstrates that elevated orexin 2 receptor (OX2R) activity within the rostral ventrolateral medulla (RVLM) contributes to hypertension in spontaneously hypertensive rats (SHRs), and a lower OX2R protein level was detected in their RVLM. The p...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/EP085016

    authors: Lee YH,Tsai MC,Li TL,Dai YW,Huang SC,Hwang LL

    更新日期:2015-09-01 00:00:00

  • Cerebral haemodynamics and oxygenation during whole-body exercise over 5 days at high altitude.

    abstract:NEW FINDINGS:What is the central question of this study? Impairment and subsequent improvement in cerebral oxygenation during acute and prolonged exposure to high altitude affect exercise performance. This study innovates by investigating the effect of acute and prolonged high-altitude exposure on cerebral haemodynamic...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/EP088354

    authors: Marillier M,Rupp T,Bouzat P,Walther G,Baillieul S,Millet GY,Robach P,Verges S

    更新日期:2021-01-01 00:00:00

  • Regulation of cerebral blood flow and metabolism during exercise.

    abstract:NEW FINDINGS:What is the topic of this review? The manuscript collectively combines the experimental observations from >100 publications focusing on the regulation of cerebral blood flow and metabolism during exercise from 1945 to the present day. What advances does it highlight? This article highlights the importance ...

    journal_title:Experimental physiology

    pub_type: 杂志文章,评审

    doi:10.1113/EP086249

    authors: Smith KJ,Ainslie PN

    更新日期:2017-11-01 00:00:00

  • New insights into central cardiovascular control during exercise in humans: a central command update.

    abstract::The autonomic adjustments to exercise are mediated by central signals from the higher brain (central command) and by a peripheral reflex arising from working skeletal muscle (exercise pressor reflex), with further modulation provided by the arterial baroreflex. Although it is clear that central command, the exercise p...

    journal_title:Experimental physiology

    pub_type: 杂志文章,评审

    doi:10.1113/expphysiol.2005.032037

    authors: Williamson JW,Fadel PJ,Mitchell JH

    更新日期:2006-01-01 00:00:00

  • Neuropeptidergic and autonomic control of the vasculature of the rat knee joint revealed by laser Doppler perfusion imaging.

    abstract::An imaging technique (laser Doppler perfusion imaging, LDI), based on measurement of backscattered Doppler-broadened laser radiation, was used to provide two-dimensional images of perfusion in the exposed rat knee joint capsule. In control animals, frequency-dependent constriction of knee joint blood vessels occurred ...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/expphysiol.1995.sp003851

    authors: Karimian SM,McDougall JJ,Ferrell WR

    更新日期:1995-05-01 00:00:00

  • House dust mite potentiates capsaicin-evoked Ca2+ transients in mouse pulmonary sensory neurons via activation of protease-activated receptor-2.

    abstract::House dust mite (HDM) is a major source of allergen in house dust and has been suggested to be involved in the pathogenesis of asthma. In this study, we aimed to investigate whether HDM can modulate the sensitivity of pulmonary sensory neurons and, if so, to elucidate the underlying mechanism. Fura-2-based ratiometric...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/expphysiol.2011.060764

    authors: Gu Q,Lee LY

    更新日期:2012-04-01 00:00:00

  • Natural killer cell mobilization and egress following acute exercise in men with prostate cancer.

    abstract:NEW FINDINGS:What is the central question of this study? What are the characteristics of the NK cell response following acute moderate-intensity aerobic exercise in prostate cancer survivors and is there a relationship between stress hormones and NK cell mobilization? What is the main finding and its importance? NK cel...

    journal_title:Experimental physiology

    pub_type: 杂志文章

    doi:10.1113/EP088627

    authors: Hanson ED,Sakkal S,Que S,Cho E,Spielmann G,Kadife E,Violet JA,Battaglini CL,Stoner L,Bartlett DB,McConell GK,Hayes A

    更新日期:2020-09-01 00:00:00