SOLUBLE ACTIVIN RECEPTOR TYPE IIB INCREASES FORWARD PULLING TENSION IN THE mdx MOUSE

被引：29

作者：

Carlson, C. George ^{[1
]}

Bruemmer, Kay ^{[1
]}

Sesti, Jenna ^{[1
]}

Stefanski, Casey ^{[1
]}

Curtis, Heather ^{[1
]}

Ucran, Jeffrey ^{[2
]}

Lachey, Jennifer ^{[2
]}

Seehra, Jasbir S. ^{[2
]}

机构：

[1] AT Still Univ, Kirksville Coll Osteopath Med, Dept Physiol, Kirksville, MO 63501 USA

[2] Acceleron Pharma, Cambridge, MA USA

来源：

MUSCLE & NERVE | 2011年 / 43卷 / 05期

基金：

美国国家卫生研究院;

关键词：

activin receptor type IIB; Duchenne muscular dystrophy; mdx mouse; ursodeoxycholic acid; whole body tension; MUSCLE DEGENERATION; UTROPHIN; STRENGTH; MICE;

D O I：

10.1002/mus.21944

中图分类号：

R74 [神经病学与精神病学];

学科分类号：

100204 [神经病学];

摘要：

Introduction: In this study we investigated the action of RAP-031, a soluble activin receptor type IIB (ActRIIB) comprised of a form of the ActRIIB extracellular domain linked to a murine Fc, and the NF-kappa B inhibitor, ursodeoxycholic acid (UDCA), on the whole body strength of mdx mice. Methods: The whole body tension (WBT) method of assessing the forward pulling tension (FPT) exerted by dystrophic (mdx) mice was used. Results: RAP-031 produced a 41% increase in body mass and a 42.5% increase in FPT without altering the FPT normalized for body mass (WBT). Coadministration of RAP-031 with UDCA produced increases in FPT that were associated with an increase in WBT. Conclusions: Myostatin inhibition increases muscle mass without altering the fundamental weakness characteristic of dystrophic muscle. Cotreatment with an NF-kappa B inhibitor potentiates the effects of myostatin inhibition in improving FPT in mdx mice. Muscle Nerve 43: 694-699, 2011

引用

页码：694 / 699

页数：6

共 21 条

[1]

Interplay of IKK/NF-κB signaling in macrophages and myofibers promotes muscle degeneration in Duchenne muscular dystrophy [J].

Acharyya, Swarnali ;

Villalta, S. Armando ;

Bakkar, Nadine ;

Bupha-Intr, Tepmanas ;

Janssen, Paul M. L. ;

Carathers, Micheal ;

Li, Zhi-Wei ;

Beg, Amer A. ;

Ghosh, Sankar ;

Sahenk, Zarife ;

Weinstein, Michael ;

Gardner, Katherine L. ;

Rafael-Fortney, Jill A. ;

Karin, Michael ;

Tidball, James G. ;

Baldwin, Albert S. ;

Guttridge, Denis C. .

JOURNAL OF CLINICAL INVESTIGATION, 2007, 117 (04) :889-901

[2]

Functional improvement of dystrophic muscle by myostatin blockade [J].

Bogdanovich, S ;

Krag, TOB ;

Barton, ER ;

Morris, LD ;

Whittemore, LA ;

Ahima, RS ;

Khurana, TS .

NATURE, 2002, 420 (6914) :418-421

[3]

A simple protocol for assessing inter-trial and inter-examiner reliability for two noninvasive measures of limb muscle strength [J].

Carlson, C. George ;

Rutter, John ;

Bledsoe, Cathy ;

Singh, Rajvir ;

Hoff, Helena ;

Bruemmer, Kay ;

Sesti, Jenna ;

Gatti, Francesca ;

Berge, Jonas ;

McCarthy, Laura .

JOURNAL OF NEUROSCIENCE METHODS, 2010, 186 (02) :226-230

[4]

Chronic treatment with agents that stabilize cytosolic IκB-α enhances survival and improves resting membrane potential in MDX muscle fibers subjected to chronic passive stretch [J].

Carlson, CG ;

Samadi, A ;

Siegel, A .

NEUROBIOLOGY OF DISEASE, 2005, 20 (03) :719-730

[5]

A NONINVASIVE PROCEDURE TO DETECT MUSCLE WEAKNESS IN THE MDX MOUSE [J].

CARLSON, CG ;

MAKIEJUS, RV .

MUSCLE & NERVE, 1990, 13 (06) :480-484

[6]

CARLSON CG, 2009, M22006 SOP

[7]

Expression of truncated utrophin leads to major functional improvements in dystrophin-deficient muscles of mice [J].

Deconinck, N ;

Tinsley, J ;

DeBacker, F ;

Fisher, R ;

Kahn, D ;

Phelps, S ;

Davies, K ;

Gillis, JM .

NATURE MEDICINE, 1997, 3 (11) :1216-1221

[8]

Force impairment in calpain 3-deficient mice is not correlated with mechanical disruption [J].

Fougerousse, F ;

Gonin, P ;

Durand, M ;

Richard, I ;

Raymackers, JM .

MUSCLE & NERVE, 2003, 27 (05) :616-623

[9]

Multivariate evaluation of the functional recovery obtained by the overexpression of utrophin in skeletal muscles of the mdx mouse [J].

Gillis, JM .

NEUROMUSCULAR DISORDERS, 2002, 12 :S90-S94

[10]

GILLIS JP, 2008, M11001 SOP

← 1 2 3 →