Department of Orthopaedics and Traumatology, SH Ho Scoliosis Research Laboratory, The Chinese University of Hong Kong, Shatin, NT, Hong Kong SAR, China.
Joint Scoliosis Research Center of the Chinese University of Hong Kong and Nanjing University, The Chinese University of Hong Kong, Shatin, NT, Hong Kong SAR, China.
Stud Health Technol Inform. 2021 Jun 28;280:14-17. doi: 10.3233/SHTI210425.
AIS is three-dimensional spinal deformity with unclear etiopathogenesis. LBX1 is so far the only multi-centers validated AIS predisposing gene. The imbalance of posterior paraspinal muscles is an important factor in AIS etiopathogenesis. It is poorly understood how LBX1 contributes to the abnormal paraspinal muscles and onset/progression of AIS. We aimed to evaluate the expression of LBX1 in paraspinal muscles at the concave and convex side in AIS, and whether alternation of LBX1 expression could affect myoblastsactivities and potentially influence muscle-bone interaction via myokines expression. Paraspinal muscles from AIS and age- and curvature-matched congenital scoliosis (CS) patients were collected for fiber types analysis. Biopsies were also subjected to qPCR to validate expression of myogenic markers, selected myokines and LBX1. Human skeletal muscle myoblast (HSMM) was used for LBX1 loss-of-function study in vitro. Muscle fiber types analysis showed type I and type IIX/IIAX fibers proportion were significantly different between AIS concave and convex but not in two sides of CS. LBX1, myogenic markers and one myokine were significantly imbalanced in AIS but not in CS. Loss-of-function study showed knockdown of LBX1 could inhibit myogenic markers expression and myokines as well. This study provides new insight into the association between imbalanced paraspinal muscle and potential muscle-bone crosstalk in AIS patients and the biological function of predisposing gene LBX1. Further investigation with appropriate animal models is warranted to explore if asymmetric expression of LBX1 could result in distinct muscle phenotypes and bone qualities thus affect the progression of spine curvature in AIS.
AIS 是一种三维脊柱畸形,其病因尚未明确。LBX1 是迄今为止唯一经过多中心验证的 AIS 易感性基因。后路脊柱旁肌失衡是 AIS 病因学的一个重要因素。目前尚不清楚 LBX1 如何导致脊柱旁肌异常以及 AIS 的发病和进展。我们旨在评估 LBX1 在 AIS 凹侧和凸侧脊柱旁肌中的表达情况,以及 LBX1 表达的改变是否会影响成肌细胞的活性,并通过肌因子的表达潜在地影响肌肉-骨骼相互作用。收集 AIS 和年龄及曲率匹配的先天性脊柱侧凸(CS)患者的脊柱旁肌进行纤维类型分析。还对活检进行 qPCR 以验证成肌标志物、选定的肌因子和 LBX1 的表达。体外使用人骨骼肌成肌细胞(HSMM)进行 LBX1 功能丧失研究。肌肉纤维类型分析显示,AIS 凹侧和凸侧的 I 型和 IIX/IIAX 型纤维比例存在显著差异,但 CS 两侧则无差异。AIS 患者的 LBX1、成肌标志物和一种肌因子明显失衡,但 CS 患者则不然。功能丧失研究表明,LBX1 的敲低可抑制成肌标志物和肌因子的表达。本研究为 AIS 患者脊柱旁肌失衡与潜在的肌肉-骨骼相互作用之间的关联以及易感基因 LBX1 的生物学功能提供了新的见解。需要进一步的研究以探索适当的动物模型,以研究 LBX1 的不对称表达是否会导致不同的肌肉表型和骨骼质量,从而影响 AIS 脊柱曲率的进展。