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Sampaolesi M Blot S D'Antona G Granger N Tonlorenzi R Innocenzi A Mognol P Thibaud JL Galvez BG Barthélémy I Perani L Mantero S Guttinger M Pansarasa O Rinaldi C Cusella De Angelis MG Torrente Y Bordignon C Bottinelli R Cossu G 《Nature》2006,444(7119):574-579
Duchenne muscular dystrophy remains an untreatable genetic disease that severely limits motility and life expectancy in affected children. The only animal model specifically reproducing the alterations in the dystrophin gene and the full spectrum of human pathology is the golden retriever dog model. Affected animals present a single mutation in intron 6, resulting in complete absence of the dystrophin protein, and early and severe muscle degeneration with nearly complete loss of motility and walking ability. Death usually occurs at about 1 year of age as a result of failure of respiratory muscles. Here we report that intra-arterial delivery of wild-type canine mesoangioblasts (vessel-associated stem cells) results in an extensive recovery of dystrophin expression, normal muscle morphology and function (confirmed by measurement of contraction force on single fibres). The outcome is a remarkable clinical amelioration and preservation of active motility. These data qualify mesoangioblasts as candidates for future stem cell therapy for Duchenne patients. 相似文献
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Parma P Radi O Vidal V Chaboissier MC Dellambra E Valentini S Guerra L Schedl A Camerino G 《Nature genetics》2006,38(11):1304-1309
R-spondins are a recently characterized small family of growth factors. Here we show that human R-spondin1 (RSPO1) is the gene disrupted in a recessive syndrome characterized by XX sex reversal, palmoplantar hyperkeratosis and predisposition to squamous cell carcinoma of the skin. Our data show, for the first time, that disruption of a single gene can lead to complete female-to-male sex reversal in the absence of the testis-determining gene, SRY. 相似文献
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Marjan Radi Behnam Dezfouli Kamalrulnizam Abu Bakar Shukor Abd Razak Mohammad Ali Nematbakhsh 《清华大学学报》2011,16(5):475-490
The existing multipath routing protocols for wireless sensor networks demonstrate the efficacy of traffic distribution over multiple paths to fulfill the Quality of Service (QoS) requirements of different applications.However,the performance of these protocols is highly affected by the characteristics of the wireless channel and may be even inferior to the performance of single-path approaches.Specifically,when multiple adjacent paths are being used concurrently,the broadcast nature of wireless channels res... 相似文献
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