Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise

Lamhonwah, Anne-Marie; Bear, Christine E.; Huan, Ling Jun; Chiaw, Patrick Kim; Ackerley, Cameron; Tein, Ingrid

doi:10.1002/ana.21982

Cited by 90 publications

(77 citation statements)

References 35 publications

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“…These findings and our current data contrast with the typical localization of CFTR to the plasma membrane of polarized epithelial and serous cells (27,33,34). We further localized CFTR to the SR in ASM cells, a finding consistent with earlier reports of CFTR localization to the SR in skeletal muscle cells (41,42). CF disease manifestations are typically related to loss of CFTR function at the plasma membrane in epithelial cells.…”

Section: /2supporting

confidence: 82%

Cystic Fibrosis Transmembrane Conductance Regulator in Sarcoplasmic Reticulum of Airway Smooth Muscle. Implications for Airway Contractility

Cook¹,

Rector²,

Bouzek³

et al. 2016

Am J Respir Crit Care Med

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Section: /2supporting

confidence: 82%

Cystic Fibrosis Transmembrane Conductance Regulator in Sarcoplasmic Reticulum of Airway Smooth Muscle. Implications for Airway Contractility

Cook¹,

Rector²,

Bouzek³

et al. 2016

Am J Respir Crit Care Med

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“…Resting muscle ATP values have not been previously reported in the CF or PCD populations. Whether the decreased ATP levels we have observed is primary (6,7,30,31) or secondary to a defect in CFTR in the muscles of patients with CF cannot be directly determined from the current data. It is important to note that the biological consequence of this relatively small difference is unclear.…”

Section: Discussionmentioning

confidence: 90%

“…A report has shown that an intrinsic alteration of function is linked to the absence of CFTR from skeletal muscle, leading to dysregulated calcium homeostasis, augmented inflammatory or atrophic gene expression signatures, and increased diaphragm muscle weakness (6). Our research team has recently demonstrated the expression of CFTR in human skeletal muscle (7). In addition, evidence suggests that impaired anaerobic performance (8), abnormal anaerobic metabolism (9), and decreased maximal muscle strength and power (10 -12) occurs in CF patients.…”

mentioning

confidence: 99%

Skeletal Muscle Metabolism in Cystic Fibrosis and Primary Ciliary Dyskinesia

et al. 2011

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Previous studies have reported differences in muscle function and metabolism between patients with cystic fibrosis (CF) and healthy controls (HC), but it is currently unknown whether these abnormalities are specific to CF or also seen in other airway diseases. In this study, we used magnetic resonance spectroscopy (MRS) during exercise to assess muscle metabolism in CF patients. Twenty patients with CF and 20 age, gender, and habitual activity-matched HCs and a respiratory disease comparison group with primary ciliary dyskinesia (PCD; n ϭ 10) were studied.31 Phosphorus MRS ( 31 P-MRS) was used to characterize muscle bioenergetic metabolism at rest and after high-, moderate-, and low-intensity exercise. CF patients exhibited lower resting ATP/phosphocreatine (PCr) ratio and significantly higher end-exercise pH values compared with both HC and PCD patients. Both CF and PCD patients demonstrated significantly slower PCr recovery time constants after high-intensity exercise. Our results suggest that not only there are specific abnormalities of muscle metabolism in CF patients but also there is a nonspecific impact of respiratory disease on muscle function. (Pediatr Res 69: 40-45, 2011)

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“…The samples were fixed in 2.5% glutaraldehyde at 4°C for 24 h and a semi-thin sectioning approach was used to locate the area of interest before the samples were prepared as ultrathin sections as described previously (12). Ultrastructure was observed using a Hitachi H600 TEM (Hitachi, Tokyo).…”

Section: Transmission Electron Microscope (Tem) Examinationmentioning

confidence: 99%

Effects of Induction/Inhibition of Endogenous Heme Oxygenase-1 on Lipid Metabolism, Endothelial Function, and Atherosclerosis in Rabbits on a High Fat Diet

Liu¹,

Wu³

et al. 2012

J Pharmacol Sci

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Abstract. The heme oxygenase-1 (HO-1) / carbon monoxide (CO) system has been presumed as a therapeutic target for preventing atherosclerosis. However, the exact mechanism(s) underlying this system remains largely undefined. This study aims to examine the influence of induction/inhibition of HO-1 on atherosclerotic plaque using pharmacological approaches and to elucidate potential mechanisms. Rabbits were randomly assigned to receive a standard diet (control group), high fat diet (HFD), HFD plus HO inducer hemin (HFD + H group), and HFD plus an HO inhibitor, zinc protoporphyrin-9 (ZnPP9, HFD + Z group). Atherosclerotic plaque was evaluated using oil red O staining and histological analyses. Immunohistochemistry, western blotting, and RT-PCR were employed to study the expression of HO-1 and endothelin-1 (ET-1). Levels of CO, nitric oxide (NO), eNOS/iNOS activities, NF-κB activity, and TNF-α level were determined. No significant differences of serum lipid levels were observed among the HFD, HFD + Z, and HFD + H groups. In rabbits, HFD induced typical atherosclerotic plaque and increased intima/media thickness ratio, which was markedly reduced in the HFD + H group and further aggravated in the HFD + Z group. Furthermore, hemin increased HO-1 expression, CO levels, and eNOS activity, while decreasing iNOS levels, ET-1 expression, NF-κB activity, and TNF-α level. ZnPP9 caused opposite effects. Induction of the endogenous HO-1/CO system by hemin can prevent atherosclerosis though increasing CO levels, regulating eNOS activity, NF-κB activity, TNF-α levels, and ET-1 levels in rabbits. Our results add new evidence for the importance of HO-1 in the genesis and development of atherosclerosis and provide several possible mechanisms underlying the antiatherosclerosis effects of HO-1.

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Cystic fibrosis transmembrane conductance regulator in human muscle: Dysfunction causes abnormal metabolic recovery in exercise

Cited by 90 publications

References 35 publications

Cystic Fibrosis Transmembrane Conductance Regulator in Sarcoplasmic Reticulum of Airway Smooth Muscle. Implications for Airway Contractility

Cystic Fibrosis Transmembrane Conductance Regulator in Sarcoplasmic Reticulum of Airway Smooth Muscle. Implications for Airway Contractility

Skeletal Muscle Metabolism in Cystic Fibrosis and Primary Ciliary Dyskinesia

Effects of Induction/Inhibition of Endogenous Heme Oxygenase-1 on Lipid Metabolism, Endothelial Function, and Atherosclerosis in Rabbits on a High Fat Diet

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