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Loss of cIAP1 attenuates soleus muscle pathology and improves diaphragm function in mdx mice


, : Loss of cIAP1 attenuates soleus muscle pathology and improves diaphragm function in mdx mice. Human Molecular Genetics 22(5): 867-878

The cellular inhibitor of apoptosis 1 (cIAP1) protein is an essential regulator of canonical and noncanonical nuclear factor κB (NF-κB) signaling pathways. NF-κB signaling is known to play important roles in myogenesis and degenerative muscle disorders such as Duchenne muscular dystrophy (DMD), but the involvement of cIAP1 in muscle disease has not been studied directly. Here, we asked whether the loss of cIAP1 would influence the pathology of skeletal muscle in the mdx mouse model of DMD. Double-mutant cIAP1(-/-);mdx mice exhibited reduced muscle damage and decreased fiber centronucleation in the soleus, compared with single-mutant cIAP1(+/+);mdx mice. This improvement in pathology was associated with a reduction in muscle infiltration by macrophages and diminished expression of inflammatory cytokines such as IL-6 and tumor necrosis factor-α. Furthermore, the cIAP1(-/-);mdx mice exhibited reduced serum creatine kinase, and improved exercise endurance associated with improved exercise resilience by the diaphragm. Mechanistically, the loss of cIAP1 was sufficient to drive constitutive activation of the noncanonical NF-κB pathway, which led to increased myoblast fusion in vitro and in vivo. Collectively, these results show that the loss of cIAP1 protects skeletal muscle from the degenerative pathology resulting from systemic loss of dystrophin.

US$19.90

PMID: 23184147

DOI: 10.1093/hmg/dds493


Other references

Gruca, A., 1953: Transplantation of the femoral hamstrings in the treatment of cerebral lesions. Chirurgia Narzadow Ruchu i Ortopedia Polska 18(4): 303-310

Mora, M.; Scarfone, A.C.lvani, M.G.eco, A.; Mingrone, G., 2003: Insulin clearance in obesity. Insulin uptake and degradation is a complex and not yet completely understood process involving not only insulin sensitive tissues. The most important degradative system is insulin degrading enzyme which is a highly conserved metalloendopeptidase...

Krenik K.D.; Kephart G.M.; Offord K.P.; Dunnette S.L.; Gleich G.J., 1989: Comparison of antifading agents used in immunofluorescence. Various chemicals including p-phenylenediamine, n-propyl gallate, 1,4-diazobicyclo[2,2,2]-octane and Citifluor were tested to determine their effectiveness in retarding the fading of fluorescein-conjugated antibody bound to eosinophil granule majo...

Cubitt, B.; de la Torre, J.C., 1997: Amantadine does not have antiviral activity against Borna disease virus. We have investigated the antiviral activity of amantadine (AD) against Borna disease virus (BDV) in several culture cell systems. We present evidence that AD, in the range 5 to 10 microM, does not have antiviral activity against BDV. Treatment of...

Freeman, P.B., 2006: Obviate. Optometry 77(10): 471-472

Suárez-Peñaranda, Jé.Manuel.; Vieites, Bña.; Evgenyeva, E.; Vázquez-Veiga, H.; Forteza, J., 2007: Male genital leiomyomas showing androgen receptor expression. Genital leiomyoma in men include those superficial leiomyomas arising in the scrotum and the areola. They are unusual neoplasms: few cases have been reported in the literature and they usually escape clinical diagnosis. Three cases of male genital...

Borell, E.M.; Romatzki, S.B.C.; Ferse, S.C.A., 2010: Differential physiological responses of two congeneric scleractinian corals to mineral accretion and an electric field. Despite increasing popularity of 'electric' reefs as a means for reef restoration, there is a distinct lack of quantitative evidence supporting the alleged benefits of this method. This study investigated the effects of an electric field...

Kaumanns, W.; Wilde, J.; Schwibbe, M.; Hindahl, J.; Klensang, H., 1988: Zur Haltung von Bartaffen (Macaca silenus). Zeitschrift des Koelner Zoo, 313: 87-106

Li, G-yuan.; Liu, Z-jian.; Chen, H-jun.; Zhang, X-hong.; Jiang, J-jie.; Hu, A-zhong., 2012: A correlation study of Tei index and N-terminal pro-brain natriuretic peptide in patients with high-altitude heart disease. To explore the diagnostic value of Tei index of right ventricle and serum level of NT-proBNP in patients with high-altitude heart disease (HAHD). Right ventricle Tei index and serum NT-proBN level were calculated and tested in 32 local healthy vol...

Kramer, H.; Jacobs, D.R.; Bild, D.; Post, W.; Saad, M.F.; Detrano, R.; Tracy, R.; Cooper, R.; Liu, K., 2005: Urine albumin excretion and subclinical cardiovascular disease. The Multi-Ethnic Study of Atherosclerosis. We examined the association between urine albumin excretion ( UAE) and common and internal carotid artery intima-media thickness ( IMT), end-diastolic left ventricular ( LV) mass, and coronary artery calcification ( CAC) scores using data from the...