The Role of Cystic Fibrosis Transmembrane Conductance Regulator in Skeletal Muscle Contractile Function

调节器 囊性纤维化 囊性纤维化跨膜传导调节器 跨膜蛋白 功能(生物学) 骨骼肌 化学 细胞生物学 生物物理学 医学 解剖 内科学 生物 生物化学 受体 基因
作者
Kenneth Wu,Anna Michalski,Jenna Sykes,Jane Batt,Anne L. Stephenson,Sunita Mathur
出处
期刊:Cardiopulmonary physical therapy journal [Ovid Technologies (Wolters Kluwer)]
标识
DOI:10.1097/cpt.0000000000000259
摘要

Purpose: Genetic mutations in cystic fibrosis (CF) result in CF transmembrane conductance regulator (CFTR) dysfunction. CFTR is expressed in human skeletal muscle; its effect on skeletal muscle abnormalities is unknown. The study objective is to investigate the role of CFTR in skeletal muscle contractile function. Methods: We conducted a prospective, cross-sectional study comparing 34 adults with minimal and 18 with residual function CFTR mutations, recruited from Toronto Adult CF Centre, St. Michael's Hospital, Unity Health Toronto. Quadriceps, biceps brachii, and handgrip strength was measured with dynamometers; leg muscle power with the stair climb power test. Quadriceps muscle contractility was determined by quadriceps muscle strength normalized to quadriceps muscle size, measured with ultrasound images. Multivariable regression was used for analysis. Results: People with residual function CFTR mutations had higher quadriceps muscle torque normalized to quadriceps layer thickness and to rectus femoris cross-sectional area by 27.5 Nm/cm [95% CI (2.2, 52.8) Nm/cm, P = .034] and 5.6 Nm/cm 2 [95% CI (0.3, 10.9) Nm/cm 2 , P = .041], respectively, compared with those with minimal function CFTR mutations. There were no differences in quadriceps muscle torque ( P = .58), leg muscle power ( P = .47), biceps brachii muscle force ( P = .14), or handgrip force ( P = .12) between the 2 mutation groups. Conclusions: CFTR protein may play a role in muscle contractility, implying a limited capacity to exert muscle force per unit of muscle size in people with CF. This suggests that building a greater muscle mass through resistance exercises focusing on muscle hypertrophy in exercise prescription may improve muscle strength in people with CF.
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