spinal muscular atrophy type II;
arm cycling exercise;
SMN protein;
SMN2 copy number;
IGF1;
IGFBP3;
MODEL MOUSE;
CHILDREN;
EPIGENOME;
SURVIVAL;
MICE;
SMA;
D O I:
10.1177/0883073817750500
中图分类号:
R74 [神经病学与精神病学];
学科分类号:
摘要:
Exercise studies in neuromuscular diseases like spinal muscular atrophy (SMA), a devastating disease caused by survival of motor neuron 1 (SMN1) gene mutations, are drawing attention due to its beneficial effects. In this study, we presented a constructed arm cycling exercise protocol and evaluated the benefits on SMA patients. Five SMA type II patients performed 12 weeks of supervised arm cycling exercise. The physical functions were evaluated together with the SMN2 copy numbers, SMN protein levels, insulin-like growth factor 1(IGF1) and binding protein 3 (IGFBP3) levels. The active cycling distance and duration of patients significantly improved. Significant changes could not have detected either SMN or IGF1 and IGFBP3 levels in response to exercise. The findings demonstrated that the patients tolerated the exercise protocol and gained a benefit from arm cycling but benefits could not be associated with SMN2 copy number, SMN protein level, IGF1, or IGFBP3 levels.
机构:
IRCCS Neuromed, Via Atinense 18, I-86077 Pozzilli, IS, ItalyUniv Pisa, Dept Clin & Expt Med, Via Roma 55, I-56126 Pisa, Italy
Biagioni, Francesca
Limanaqi, Fiona
论文数: 0引用数: 0
h-index: 0
机构:
Univ Pisa, Dept Translat Res & New Technol Med & Surg, Via Roma 55, I-56126 Pisa, ItalyUniv Pisa, Dept Clin & Expt Med, Via Roma 55, I-56126 Pisa, Italy
Limanaqi, Fiona
Busceti, Carla L.
论文数: 0引用数: 0
h-index: 0
机构:
IRCCS Neuromed, Via Atinense 18, I-86077 Pozzilli, IS, ItalyUniv Pisa, Dept Clin & Expt Med, Via Roma 55, I-56126 Pisa, Italy
Busceti, Carla L.
Ryskalin, Larisa
论文数: 0引用数: 0
h-index: 0
机构:
Univ Pisa, Dept Translat Res & New Technol Med & Surg, Via Roma 55, I-56126 Pisa, ItalyUniv Pisa, Dept Clin & Expt Med, Via Roma 55, I-56126 Pisa, Italy
Ryskalin, Larisa
Lenzi, Paola
论文数: 0引用数: 0
h-index: 0
机构:
Univ Pisa, Dept Translat Res & New Technol Med & Surg, Via Roma 55, I-56126 Pisa, ItalyUniv Pisa, Dept Clin & Expt Med, Via Roma 55, I-56126 Pisa, Italy
Lenzi, Paola
Fornai, Francesco
论文数: 0引用数: 0
h-index: 0
机构:
IRCCS Neuromed, Via Atinense 18, I-86077 Pozzilli, IS, Italy
Univ Pisa, Dept Translat Res & New Technol Med & Surg, Via Roma 55, I-56126 Pisa, ItalyUniv Pisa, Dept Clin & Expt Med, Via Roma 55, I-56126 Pisa, Italy
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Univ Wisconsin, Dept Orthoped & Rehabil, Madison, WI 53706 USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Hanna, Rewais B.
Nahm, Nick
论文数: 0引用数: 0
h-index: 0
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
UNMC Childrens Hosp & Med Ctr, Omaha, NE 68114 USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Nahm, Nick
Bent, Melissa A.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Univ Wisconsin, Dept Orthoped & Rehabil, Madison, WI 53706 USA
Childrens Hosp Los Angeles, Los Angeles, CA USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Bent, Melissa A.
Sund, Sarah
论文数: 0引用数: 0
h-index: 0
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Univ Wisconsin, Dept Orthoped & Rehabil, Madison, WI 53706 USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Sund, Sarah
Patterson, Karen
论文数: 0引用数: 0
h-index: 0
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Univ Wisconsin, Dept Phys Therapy, Madison, WI USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Patterson, Karen
Schroth, Mary K.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Cure SMA, Elk Grove Village, IL USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Schroth, Mary K.
Halanski, Matthew A.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Univ Wisconsin, Dept Orthoped & Rehabil, Madison, WI 53706 USA
UNMC Childrens Hosp & Med Ctr, Omaha, NE 68114 USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Li, Dan
Sun, Na
论文数: 0引用数: 0
h-index: 0
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Sun, Na
Xiang, Li
论文数: 0引用数: 0
h-index: 0
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Neurol, Xian, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Xiang, Li
Liu, Jingjie
论文数: 0引用数: 0
h-index: 0
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Neurol, Xian, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Liu, Jingjie
Wang, Xueying
论文数: 0引用数: 0
h-index: 0
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Wang, Xueying
Yang, Lin
论文数: 0引用数: 0
h-index: 0
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, 157 Xiwu Rd, Xian 710004, Shaanxi, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Yang, Lin
Huang, Shaoping
论文数: 0引用数: 0
h-index: 0
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, 157 Xiwu Rd, Xian 710004, Shaanxi, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Huang, Shaoping
[J].
DRUG DESIGN DEVELOPMENT AND THERAPY,
2024,
18
: 953
-
965
机构:
IRCCS Neuromed, Via Atinense 18, I-86077 Pozzilli, IS, ItalyUniv Pisa, Dept Clin & Expt Med, Via Roma 55, I-56126 Pisa, Italy
Biagioni, Francesca
Limanaqi, Fiona
论文数: 0引用数: 0
h-index: 0
机构:
Univ Pisa, Dept Translat Res & New Technol Med & Surg, Via Roma 55, I-56126 Pisa, ItalyUniv Pisa, Dept Clin & Expt Med, Via Roma 55, I-56126 Pisa, Italy
Limanaqi, Fiona
Busceti, Carla L.
论文数: 0引用数: 0
h-index: 0
机构:
IRCCS Neuromed, Via Atinense 18, I-86077 Pozzilli, IS, ItalyUniv Pisa, Dept Clin & Expt Med, Via Roma 55, I-56126 Pisa, Italy
Busceti, Carla L.
Ryskalin, Larisa
论文数: 0引用数: 0
h-index: 0
机构:
Univ Pisa, Dept Translat Res & New Technol Med & Surg, Via Roma 55, I-56126 Pisa, ItalyUniv Pisa, Dept Clin & Expt Med, Via Roma 55, I-56126 Pisa, Italy
Ryskalin, Larisa
Lenzi, Paola
论文数: 0引用数: 0
h-index: 0
机构:
Univ Pisa, Dept Translat Res & New Technol Med & Surg, Via Roma 55, I-56126 Pisa, ItalyUniv Pisa, Dept Clin & Expt Med, Via Roma 55, I-56126 Pisa, Italy
Lenzi, Paola
Fornai, Francesco
论文数: 0引用数: 0
h-index: 0
机构:
IRCCS Neuromed, Via Atinense 18, I-86077 Pozzilli, IS, Italy
Univ Pisa, Dept Translat Res & New Technol Med & Surg, Via Roma 55, I-56126 Pisa, ItalyUniv Pisa, Dept Clin & Expt Med, Via Roma 55, I-56126 Pisa, Italy
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Univ Wisconsin, Dept Orthoped & Rehabil, Madison, WI 53706 USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Hanna, Rewais B.
Nahm, Nick
论文数: 0引用数: 0
h-index: 0
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
UNMC Childrens Hosp & Med Ctr, Omaha, NE 68114 USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Nahm, Nick
Bent, Melissa A.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Univ Wisconsin, Dept Orthoped & Rehabil, Madison, WI 53706 USA
Childrens Hosp Los Angeles, Los Angeles, CA USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Bent, Melissa A.
Sund, Sarah
论文数: 0引用数: 0
h-index: 0
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Univ Wisconsin, Dept Orthoped & Rehabil, Madison, WI 53706 USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Sund, Sarah
Patterson, Karen
论文数: 0引用数: 0
h-index: 0
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Univ Wisconsin, Dept Phys Therapy, Madison, WI USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Patterson, Karen
Schroth, Mary K.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Cure SMA, Elk Grove Village, IL USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Schroth, Mary K.
Halanski, Matthew A.
论文数: 0引用数: 0
h-index: 0
机构:
Univ Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
Univ Wisconsin, Dept Orthoped & Rehabil, Madison, WI 53706 USA
UNMC Childrens Hosp & Med Ctr, Omaha, NE 68114 USAUniv Wisconsin, Amer Family Childrens Hosp, Madison, WI 53706 USA
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Li, Dan
Sun, Na
论文数: 0引用数: 0
h-index: 0
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Sun, Na
Xiang, Li
论文数: 0引用数: 0
h-index: 0
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Neurol, Xian, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Xiang, Li
Liu, Jingjie
论文数: 0引用数: 0
h-index: 0
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Neurol, Xian, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Liu, Jingjie
Wang, Xueying
论文数: 0引用数: 0
h-index: 0
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Wang, Xueying
Yang, Lin
论文数: 0引用数: 0
h-index: 0
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, 157 Xiwu Rd, Xian 710004, Shaanxi, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Yang, Lin
Huang, Shaoping
论文数: 0引用数: 0
h-index: 0
机构:
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Xi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, 157 Xiwu Rd, Xian 710004, Shaanxi, Peoples R ChinaXi An Jiao Tong Univ, Affiliated Hosp 2, Dept Pediat, Xian, Peoples R China
Huang, Shaoping
[J].
DRUG DESIGN DEVELOPMENT AND THERAPY,
2024,
18
: 953
-
965