Reduced satellite cell population may lead to contractures in children with cerebral palsy.

Reduced satellite cell population may lead to contractures in children with cerebral palsy.
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卫星细胞种群减少可能会导致脑瘫儿童的闭合。

DOI:
10.1111/dmcn.12027
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发表时间:
2013-03
影响因子:
3.8
通讯作者:
Lieber RL
Lieber RL
中科院分区:
医学2区
文献类型:
--
作者:
Smith LR;Chambers HG;Lieber RL

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卫星细胞是存在于肌肉中的干细胞,负责骨骼肌的生长和修复。脑性瘫痪(CP)患者的骨骼肌纵向生长受损,导致肌肉挛缩。我们假设,卫星细胞的人口将减少在挛缩的肌肉。我们比较了患有CP挛缩的参与者(n=8; 6名男性,2名女性;年龄范围6- 15岁;粗大运动功能分类系统[GMFCS]水平II-V; 4名偏瘫,4名双瘫)和典型发育参与者(n=8; 6名男性,2名女性,年龄范围15- 18岁)的腘绳肌中的卫星细胞群。从股薄肌和半腱肌中提取肌肉活检组织,分离单核细胞。细胞表面标记物用荧光缀合抗体染色以标记卫星细胞(神经细胞粘附分子)以及炎性细胞和内皮细胞(分别为CD 34和CD 4)。使用流式细胞术分析细胞以确定细胞群。在对完整细胞进行门控后,在典型发育的儿童中平均12.8%(SD 2.8%)被确定为卫星细胞,但在CP儿童中仅为5.3%(SD 2.3%;p<0.05)。正常发育儿童和CP儿童的造血细胞和内皮细胞类型相当(p>0.05),表明分离程序是有效的。卫星细胞数量减少可能是CP中肌肉纵向生长减少的原因,其发展为固定性挛缩或CP中增强肌肉的能力降低。这表明了一种独特的肌肉骨骼疾病机制,并为使人衰弱的肌肉挛缩提供了潜在的治疗靶点。
Satellite cells are the stem cells residing in muscle responsible for skeletal muscle growth and repair. Skeletal muscle in cerebral palsy (CP) has impaired longitudinal growth that results in muscle contractures. We hypothesized that the satellite cell population would be reduced in contractured muscle. We compared the satellite cell populations in hamstring muscles from participants with CP contracture (n=8; six males, two females; age range 6–15y; Gross Motor Function Classification System [GMFCS] levels II–V; 4 with hemiplegia, 4 with diplegia) and from typically developing participants (n=8; six males, two females, age range 15–18y). Muscle biopsies were extracted from the gracilis and semitendinosus muscles and mononuclear cells were isolated. Cell surface markers were stained with fluorescently conjugated antibodies to label satellite cells (neural cell adhesion molecule) and inflammatory and endothelial cells (CD34 and CD4 respectively). Cells were analyzed using flow cytometry to determine cell populations. After gating for intact cells a mean of 12.8% (SD 2.8%) were determined to be satellite cells in typically developing children, but only 5.3% (SD 2.3%;p<0.05) in children with CP. Hematopoietic and endothelial cell types were equivalent in typically developing children and children with CP (p>0.05) suggesting the isolation procedure was valid. A reduced satellite cell population may account for the decreased longitudinal growth of muscles in CP that develop into fixed contractures or the decreased ability to strengthen muscle in CP. This suggests a unique musculoskeletal disease mechanism and provides a potential therapeutic target for debilitating muscle contractures.
DOI: 10.1007/978-1-60761-063-2_11
发表时间: 2010-01-01
期刊: PROTOCOLS FOR ADULT STEM CELLS
影响因子: --
作者:
Conboy, Michael J.;Cerletti, Massimiliano;Conboy, Irina M.
通讯作者: Conboy, Irina M.
DOI: 10.1113/jphysiol.2010.203364
发表时间: 2011-05-15
影响因子: 5.5
作者:
Smith, Lucas R.;Lee, Ki S.;Lieber, Richard L.
通讯作者: Lieber, Richard L.
DOI: 10.1002/mus.10036
发表时间: 2002-02-01
期刊: MUSCLE & NERVE
影响因子: 3.4
作者:
Lieber, RL;Fridén, J
通讯作者: Fridén, J
DOI: 10.1002/mus.880060807
发表时间: 1983-01-01
期刊: MUSCLE & NERVE
影响因子: 3.4
作者:
GIBSON, MC;SCHULTZ, E
通讯作者: SCHULTZ, E
DOI: 10.1113/jphysiol.2010.190876
发表时间: 2010-09-01
影响因子: 5.5
作者:
McKay, Bryon R.;Toth, Kyle G.;Parise, Gianni
通讯作者: Parise, Gianni