Estudos têm mostrado que o treinamento de força de baixa intensidade com oclusão vascular (TFOV) tem apresentado resultados similares nos ganhos de força e hipertrofia comparado ao treinamento de força (TF) de alta intensidade. O objetivo deste estudo foi comparar os efeitos de três diferentes programas de TF nos ganhos de força e hipertrofia musculares e na expressão da miostatina (MSTN) e seus antagonistas. Para isso, vinte e nove jovens do sexo masculino, sem experiência em TF, foram recrutados e divididos randomicamente nos grupos: treinamento de força de baixa intensidade sem oclusão (BI), treinamento de força de baixa intensidade com oclusão (BIO) e treinamento de força de alta intensidade sem oclusão (AI). Os grupos BIO e BI treinaram com intensidade de 20% 1RM, enquanto o grupo AI treinou com intensidade de 80% 1RM. A ANOVA one way foi utilizada para testar as diferenças percentuais nos ganhos de força (1RM) e na área de secção transversa (AST) do músculo quadríceps femoral. O modelo misto para análise das medidas repetidas foi utilizado para testar as diferenças nas variáveis miostatina (MSTN), folistatina-3 (FLST-3), SMAD-7 e GASP-1 nos grupos BI, BIO e AI nas condições pré e pós-treinamento. Os resultados mostraram que os aumentos de força e hipertrofia musculares nos grupos BIO e AI foram similares, entretanto superiores ao grupo BI. Esses resultados podem ser atribuídos a maior diminuição na expressão da MSTN nos grupos BIO (45%) e AI (41%) comparados com o grupo BI (16%) e o aumento na expressão dos genes que antagonizam sua atividade (SMAD-7, FLST-3 e GASP-1). Podemos concluir que a inibição na atividade da MSTN dos grupos BIO e AI podem responder em parte a similaridade nos ganhos de força e hipertrofia entre os grupos e a diferença para o grupo BI / It has been demonstrated that low intensity training associated to vascular occlusion (LIO) promotes similar gains in strength and muscle mass when compared to high intensity strength training (HI). The aim of the present study was to evaluate the effect of three different training programs on skeletal muscle hypertrophy and atrophy related gene expression. Twenty nine young male, with no previous experience in strength training were randomly allocated in three groups: low intensity strength training (i.e. 20% - 1-RM) (LI); low intensity strength training associated to vascular occlusion (i.e. 20% - 1-RM) (LIO); high intensity strength training (HI) (i.e. 80% - 1-RM). One-way ANOVA was used to assess differences in % delta change values of 1-RM and cross sectional area (CSA) of the quadriceps femoris. Mixed model analysis was used to compare myostatin (MSTN), folistatyn-3 (FLST-3), SMAD-7 e GASP-1 changes between groups pre and post training. Results demonstrated similar increases in strength and muscle hypertrophy for LIO and HI groups. Moreover, such increases were significantly greater when compared to LI. These results may be, at least in part, explained by a significant decrease in MSTN mRNA expression in LIO (45%) and HI (41%) when compared to LI (16%); additionally, SMAD-7; FLST-3 and GASP-1 mRNA expression were significantly increased. In conclusion, LIO training promotes similar gains than HI training. The results may be explained by changes in MSTN and related genes mRNA expression

In order to investigate the total number, relative proportions, size variations and distribution patterns of fibres of different types in human skeletal muscles, a technique was developed which allows whole muscle cross-sections to be collected and stained enzyme histochemically. Necropsy material was obtained from m. tibialis anterior and m. vastus lateralis of previously healthy young adults who had suffered sudden accidental deaths. The muscle specimens were extirpated within 72 hours post morten, frozen in liquid nitrogen, embedded in carboxymethylcellu- lose, sectioned and enzyme histochemically stained for myofibrillar ATPase in order to permit light microscopic identification of type 1 and type 2 muscle fibres. The present results show that there is a large variation in both the total number of fibres and the size of the whole muscle cross-sectional area between different individuals and, also, between different levels of the same muscle. The distribution of fibres of different types over the muscle cross-section was heterogeneous. Usually the relative proportion of type 2 fibres showed a peak along a medio- lateral line passing through the centre of the muscle cross-section. When comparing the left and right m. tibialis anterior of the same individual, one of the- muscles (usually the left one) was found to contain a larger number of fibres than the contralateral one. However, the pattern of distribution of fibres of different types was similar in the two muscles. The fibre sizes were also found to vary between different regions of the muscle cross-section. Both type 1 and type 2 fibres were significantly larger in the deep muscle region compared to the central or superficial sites. The mean fibre size as well as the total number of fibres correlated strongly with the whole muscle cross-sectional area. The female m. tibialis anterior contained fewer and smaller fibres than the corresponding male muscle, although the whole muscle cross-sectional area was of similar magnitude. The distribution of fibre types over the muscle cross-section differed somewhat between females and males. The variation in fibre sizes between different muscle sites was less pronounced in the females, but as in male muscles the type 2 fibres were always larger than the type 1 fibres. In conclusion, systematic variations in fibre type distribution and fibre size occurs over the muscle cross-section. The total number of fibres in the cross- section seems to vary with individual, sex, distance from muscle origin and left- right leg. The combination of fewer and smaller fibres in females compared to males leads to about 40 % smaller total muscle fibre cross-sectional area in female m. tibialis anterior. The results are discussed in relation to previous studies using muscle biopsy techniques. The possible contibution of variations in number, sizes or distribution patterns of muscle fibres to adaptation of muscle to varying functional demands is also discussed. / <p>S. 1-27: sammanfattning, s. 28-91: 5 uppsatser</p> / digitalisering@umu.se

Estudos têm mostrado que o treinamento de força de baixa intensidade com oclusão vascular (TFOV) tem apresentado resultados similares nos ganhos de força e hipertrofia comparado ao treinamento de força (TF) de alta intensidade. O objetivo deste estudo foi comparar os efeitos de três diferentes programas de TF nos ganhos de força e hipertrofia musculares e na expressão da miostatina (MSTN) e seus antagonistas. Para isso, vinte e nove jovens do sexo masculino, sem experiência em TF, foram recrutados e divididos randomicamente nos grupos: treinamento de força de baixa intensidade sem oclusão (BI), treinamento de força de baixa intensidade com oclusão (BIO) e treinamento de força de alta intensidade sem oclusão (AI). Os grupos BIO e BI treinaram com intensidade de 20% 1RM, enquanto o grupo AI treinou com intensidade de 80% 1RM. A ANOVA one way foi utilizada para testar as diferenças percentuais nos ganhos de força (1RM) e na área de secção transversa (AST) do músculo quadríceps femoral. O modelo misto para análise das medidas repetidas foi utilizado para testar as diferenças nas variáveis miostatina (MSTN), folistatina-3 (FLST-3), SMAD-7 e GASP-1 nos grupos BI, BIO e AI nas condições pré e pós-treinamento. Os resultados mostraram que os aumentos de força e hipertrofia musculares nos grupos BIO e AI foram similares, entretanto superiores ao grupo BI. Esses resultados podem ser atribuídos a maior diminuição na expressão da MSTN nos grupos BIO (45%) e AI (41%) comparados com o grupo BI (16%) e o aumento na expressão dos genes que antagonizam sua atividade (SMAD-7, FLST-3 e GASP-1). Podemos concluir que a inibição na atividade da MSTN dos grupos BIO e AI podem responder em parte a similaridade nos ganhos de força e hipertrofia entre os grupos e a diferença para o grupo BI / It has been demonstrated that low intensity training associated to vascular occlusion (LIO) promotes similar gains in strength and muscle mass when compared to high intensity strength training (HI). The aim of the present study was to evaluate the effect of three different training programs on skeletal muscle hypertrophy and atrophy related gene expression. Twenty nine young male, with no previous experience in strength training were randomly allocated in three groups: low intensity strength training (i.e. 20% - 1-RM) (LI); low intensity strength training associated to vascular occlusion (i.e. 20% - 1-RM) (LIO); high intensity strength training (HI) (i.e. 80% - 1-RM). One-way ANOVA was used to assess differences in % delta change values of 1-RM and cross sectional area (CSA) of the quadriceps femoris. Mixed model analysis was used to compare myostatin (MSTN), folistatyn-3 (FLST-3), SMAD-7 e GASP-1 changes between groups pre and post training. Results demonstrated similar increases in strength and muscle hypertrophy for LIO and HI groups. Moreover, such increases were significantly greater when compared to LI. These results may be, at least in part, explained by a significant decrease in MSTN mRNA expression in LIO (45%) and HI (41%) when compared to LI (16%); additionally, SMAD-7; FLST-3 and GASP-1 mRNA expression were significantly increased. In conclusion, LIO training promotes similar gains than HI training. The results may be explained by changes in MSTN and related genes mRNA expression

Research data indicates a persistence of elevated muscle glycogen concentration 3 days post-supercompensation in resting athletes. This study expands our earlier findings by determining whether muscle glycogen remains elevated 3, 5, or 7 days post-supercompensation. Seventeen trained male cyclists underwent one bout of exhaustive exercise to deplete muscle glycogen. This was followed by a 3-day consumption of a high carbohydrate/low protein/low fat diet (85:08:07%). Three post-loading phases followed with subjects randomly assigned to either a 3-day, 5-day, or 7-day post-loading maintenance diet of 60% carbohydrate and limited physical activity. Biopsies (50-150 mg) of the vastus lateralis were obtained pre-load (BASELINE), at peak-load (PEAK), and either at 3-day, 5-day, or 7-day post-load (POST). On average, PEAK to POST muscle glycogen concentrations decreased 34, 20 and 46% respectively for the 3-, 5-, and 7-day POST groups. Only the 7-day post-load group's PEAK to POST mean muscle glycogen concentration decreased significantly. In addition, multi-regression analysis indicated that the PEAK glycogen level was the main determinant of the number of days that glycogen levels remained significantly greater than BASELINE. Thus, trained athletes-supercompensated glycogen levels can remain higher than normal for up to 5 days post-loading. The amount of carbohydrate consumed, the level of physical activity, and the magnitude of the glycogen supercompensation determine the interval for which the glycogen levels are elevated.

The purpose of this investigation was to determine the effects of a four day creatine load and simultaneous energy restriction on muscle creatine content, exercise performance, and body composition in 24 male recreational resistance trainers, age 18-26. Sixteen subjects were randomly divided into placebo (Pl, n=8) and creatine supplement (CrS, n=8) groups. Control (C, n=8) subjects of the same age were recruited separately g à d-1 to complete the performance and body composition tests while consuming their normal diet. The CrS group was administered 20 g à d-1 of creatine monohydrate (Cr) mixed with 5 g à d-1 of sucrose, while the Pl group was administered 25 of sucrose. Both CrS and Pl consumed a formula diet of 75.3 kJ (18 kcal) à kg-1 à d-1 for 4 d. Testing before and after energy restriction consisted of a repeated sprint cycle performance test (10 sprints of 6s, with 30s rest), hydrostatic weighing, and resting needle muscle biopsy. Testing revealed that subjects in CrS and Pl demonstrated significant decreases in body weight and % body fat (%BF) with no difference between groups. However, Pl demonstrated a significantly greater % loss in FFM (2.4 ± 0.25%) compared to CrS (1.4 ± 0.4%) (p<0.05). The muscle fuel stores of CrS and Pl responded significantly to the diet. Significant increases in muscle total Cr (p<0.01), free Cr (p<0.01), and CrP (p<0.05) of 16.5%, 16.8%, and 16% respectively were demonstrated by CrS over the energy restriction period, while Pl demonstrated significant decreases of 7.2% and 8.2% respectively in muscle total Cr (p<0.01) and free Cr (p<0.05). There were no significant differences between groups for performance during the cycle test, however, there were trends toward group by time interactions for performance enhancement in CrS relative to Pl, as total work (p=0.078) and work capacity (p=0.058) increased 3.8 ± 2.2% in CrS and decreased 0.5 ± 0.4% in Pl. It was concluded that short-term energy restriction resulted in decreased muscle Cr storage, and that Cr supplementation during energy restriction increased muscle Cr and CrP stores. Consumption of Cr allowed CrS to lose a significantly lower % FFM compared to Pl. Cr supplementation resulted in trends toward improved performance in CrS relative to Pl after energy restriction, but did not influence losses in body weight or %BF. / Master of Science

Eight competitive cyclists performed two cycling trials, one following a high carbohydrate diet (H-CHO) and the other following a low carbohydrate diet (L-CHO). Trials consisted of repeated 60s maximal effort sprints to fatigue at a workload designed to elicit 125-135% VO_2peak at 90rpm. Three min of recovery separated sprints. Muscle biopsies taken at rest (biopsy 1), 85% max interval rpm (biopsy 2), and 70% max interval rpm (biopsy 3) revealed a main effect of diet on muscle glycogen levels: 609 ± 38 HCHO vs. 390 ± 42 mmol/kgdw L-CHO at biopsy 1, 383 ± 29 vs. 252 ± 28 mmol/kgdw at biopsy 2, and 346 ± 29 vs. 196 ± 18 mmol/kgdw at biopsy 3 (p<0.01). Similar decreases in muscle glycogen (45%), creatine phosphate (CP) (35%), and sarcoplasmic reticulum (SR) Ca²⁺-uptake (56%) were shown in both trials from biopsy 1 to 3. SR Ca²⁺-release decreased by 53% in H-CHO subjects and 36% in L-CHO subjects. Total exercise time tended to be longer in H-CHO than L-CHO subjects (57.5 ± 10 vs. 42.0 ± .89min) (p=0.09). H-CHO subjects exercised significantly longer than L-CHO subjects from biopsy 2 to 3 (33.6 ± 10 vs. 18 ± 3.6min) (p< 0.05). Results suggest that fatigue from 40- 60min of intermittent 60s high intensity cycling intervals is associated with reductions in muscle glycogen, CP, and SR function, and that the latter part of performance is impaired by low muscle glycogen. These data do not support a relationship between muscle glycogen status and SR function in intermittent high intensity exercise. / Master of Science

INTRODUÇÃO: Atualmente ainda não está claro se a atividade muscular e o treinamento físico afetam a síntese de carnosina muscular (Mcar) em resposta à suplementação de beta-alanina. OBJETIVO: verificar o impacto da atividade e inatividade muscular sobre o conteúdo de carnosina muscular (Mcarn) e sobre o aumento de Mcarn em resposta à suplementação de beta-alanina e, adicionalmente, verificar seu efeito sobra a capacidade de realizar esforços de alta intensidade. METHODS: Dezesseis homens treinados com lesão medular (LM) (escala ASIA: AIS A ou AIS B) foram divididos em 2 grupos: beta-alanina (BA) (N = 11) e placebo (PL) (N = 5). Amostras de biópsias musculares foram obtidas do músculo vasto lateral inativo e do deltoide ativo, antes e após 28 dias de suplementação com beta-alanina (6.4 g.dia-1). Teste t independente foi aplicado para comparar a Mcar basal e a variação absoluta (PÓS - PRÉ) entre os músculos vasto lateral e deltoide. Um teste supramáximo de carga constante até a exaustão e um teste de Wingate de série única foram aplicados para verificar a capacidade de realizar esforços de alta intensidade. Análises por Modelo Misto foram aplicadas para comparar a Mcar, o tempo até a exaustão e os valores de potência pico intra e entre sujeitos. RESULTADOS: (média ± desvio padrão): a concentração basal de Mcar no vasto lateral foi significativamente maior que no deltoide (32,0 ± 12 vs. 20,5 ± 6,1 mmol.kg-1 de músculo seco; p = 0,02). As variações absolutas na Mcar foram significativamente maiores no grupo BA em comparação com o PL, tanto para o vasto lateral (BA: 17,6 ± 10,4 mmol.kg-1 de músculo seco; PL: 2,5 ± 2,3 mmol.kg-1 de músculo seco; p = 0,002) como para o deltoide (BA: 15,7 ± 6,8 mmol.kg-1 de músculo seco; PL: 1,4 ± 2,7 mmol.kg-1 de músculo seco; p <0,001). As variações absolutas verificadas no vasto lateral e deltoide não foram diferentes entre si (vasto lateral: 17,6 ± 10,4; deltóide: 15,7 ± 6,8 mmol.kg-1 do músculo seco; p = 0,6). Não houve efeito da suplementação sobre o tempo até exaustão e potência pico. CONCLUSÃO: a inatividade muscular crônica ocasionada por lesão medular, não afeta a Mcarn e também não interfere sobre o aumento de Mcarn em resposta à suplementação de BA. Estes resultados sugerem que a atividade muscular ou o estado de treinamento não influenciam a capacidade de síntese de Mcarn em resposta à suplementação de beta-alanina / BACKGROUND: it is currently unclear whether muscle activity and exercise training affect the ability of the skeletal muscle to synthesise carnosine in response to beta-alanine supplementation. PURPOSE: to study the impact of the extremes of muscle activity and inactivity on muscle carnosine content (Mcarn) and Mcarn loading in response to beta-alanine supplementation. METHODS: 16 trained male with spinal cord injury (SCI) (ASIA scale: AIS A or AIS B) were divided into 2 groups: beta-alanine (BA) (N = 11) and placebo (PL) (N = 5). Muscle biopsies samples were obtained from active deltoid and paralysed vastus lateralis at baseline and after 28 days of &#946;-alanine supplementation (6.4 g.day-1). Unpaired t-tests were applied to compare Mcarn at baseline and the absolute pre-post change in vastus lateralis and deltoid. Mixed model was used to compare Mcarn values within- and between-subjects. RESULTS: (mean±SD): Baseline Mcarn concentration in vastus lateralis was significantly higher than in deltoid (32.0±12 vs. 20.5±6.1 mmol.kg-1 dry muscle; p=0.02). Absolute changes in Mcarn was significantly higher in the BA group in comparison with PL for both vastus lateralis (BA: 17.6±10.4 mmol.kg-1 dry muscle; PL: 2.5±2.3 mmol.kg-1 dry muscle; p=0.002) and deltoid (BA: 15.7±6.8 mmol?kg-1 dry muscle; PL: 1.4±2.7 mmol?kg-1 dry muscle; p<0.001). Absolut changes in Mcarn following BA supplementation between inactive vastus lateralis and active deltoid was not different (vastus lateralis: 17.6±10.4; deltoid: 15.7±6.8 mmol?kg-1 dry muscle; p=0.6). CONCLUSION: chronic muscle inactivity due to paralysis in SCI does not affect Mcarn at baseline and does not affect Mcarn loading in response to BA supplementation. These results suggest that muscle activity or training status does not influence Mcarn synthesis capacity in response to beta-alanine supplementation

Sleep apnea syndrome, or sleep apneic syndrome, is a serious illness that causes a high risk of cardiovascular disease development in patients. This disease is characterized by a breathless breathing disorder and falls into a class of disorder that accompanies sleep disturbances. Sleep apnea syndrome (SAS) affects 5-15% of the population, and 50-80% of patients with type 2 diabetes mellitus (T2DM) or severe obesity. SAS has a causal contribution to the development of disorders in glucose metabolism and T2DM. Diabetes mellitus type 2 is a complex metabolic disorder in which the organism is unable to process glucose as under normal physiological conditions due to a relative insulin deficiency and simultaneous peripheral insulin resistance. Insulin resistance is eventually compensated for by increased insulin secretion, which leads to the development of hyperglycemia after failure of this compensation. T2DM is very often associated with the presence of obesity, arterial hypertension, dyslipidemia and hyperuricemia. The aim of this study is to determine if the presence of SAS in non-diabetic subjects and patients with type 2 diabetes mellitus leads to disorders in the metabolism of fatty acids in the skeletal muscle. The results of the study contribute to the understanding of the molecular mechanisms...

Before major athletic events, a taper is often prescribed to facilitate recovery and enhance performance. However, it is unknown which taper model is most effective for peaking maximal strength and positively augmenting skeletal muscle. Thus, the purpose of this study was to compare performance outcomes and skeletal muscle adaptations following a step vs. an exponential taper in strength athletes. Sixteen powerlifters (24.0 ± 4.0 years, 174.4 ± 8.2 cm, 89.8 ± 21.4 kg) participated in a 6-week training program aimed at peaking maximal strength on back squat [initial 1-repetition-maximum (1RM): 174.7 ± 33.4 kg], bench press (118.5 ± 29.9 kg), and deadlift (189.9 ± 41.2 kg). Powerlifters were matched based on relative maximal strength, and randomly assigned to either (a) 1-week overreach and 1-week step taper or (b) 1-week overreach and 3-week exponential taper. Athletes were tested pre- and post-training on measures of body composition, jumping performance, isometric squat, and 1RM. Whole muscle size was assessed at the proximal, middle, and distal vastus lateralis using ultrasonography and microbiopsies at the middle vastus lateralis site. Muscle samples ( = 15) were analyzed for fiber size, fiber type [myosin-heavy chain (MHC)-I, -IIA, -IIX, hybrid-I/IIA] using whole muscle immunohistochemistry and single fiber dot blots, gene expression, and microRNA abundance. There were significant main time effects for 1RM squat ( < 0.001), bench press ( < 0.001), and deadlift, ( = 0.024), powerlifting total ( < 0.001), Wilks Score ( < 0.001), squat jump peak-power scaled to body mass ( = 0.001), body mass ( = 0.005), fat mass ( = 0.002), and fat mass index ( = 0.002). There were significant main time effects for medial whole muscle cross-sectional area (mCSA) ( = 0.006) and averaged sites ( < 0.001). There was also a significant interaction for MHC-IIA fiber cross-sectional area (fCSA) ( = 0.014) with comparisons revealing increases following the step-taper only ( = 0.002). There were significant main time effects for single-fiber MHC-I% ( = 0.015) and MHC-IIA% ( = 0.033), as well as for MyoD ( = 0.002), MyoG ( = 0.037), and miR-499a ( = 0.033). Overall, increases in whole mCSA, fCSA, MHC-IIA fCSA, and MHC transitions appeared to favor the step taper group. An overreach followed by a step taper appears to produce a myocellular environment that enhances skeletal muscle adaptations, whereas an exponential taper may favor neuromuscular performance.

Le muscle strié squelettique est un tissu richement vascularisé. Au delà de l'apport en oxygène et en nutriments, de nouvelles fonctions des vaisseaux ont été récemment identifiées, par le biais des interactions établies entre les cellules du vaisseau (cellules endothéliales) et les cellules du muscle, en particulier les cellules souches musculaires (cellules satellites). Celles-ci interagissent étroitement avec les cellules endothéliales pour leur expansion et leur différenciation, puis avec les cellules péri-endothéliales pour leur auto-renouvellement et leur retour à la quiescence. Les vaisseaux participent ainsi au contrôle de l’homéostasie du muscle squelettique. Grâce à ces interactions, les cellules vasculaires jouent donc un rôle central dans le remodelage tissulaire après un phénomène destructif, survenant par exemple au cours d’un trauma ou d’une myopathie. Pour étudier, les mécanismes de la plasticité vasculaire au cours du remodelage tissulaire, deux situations paradigmatiques de muscle en régénération chez l’enfant : la dermatomyosite juvénile (DMJ) et la dystrophie musculaire de Duchenne (DMD) ont été étudiées. Il existe, dans ces deux pathologies une souffrance musculaire associée à des cycles de nécrose/régénération. Elles se différencient par leur plasticité vasculaire et par leur évolution. En effet, la DMJ, la myopathie inflammatoire la plus fréquente de l’enfant est caractérisée par une vasculopathie avec perte en capillaires. L’évolution peut être favorable avec restitution ad integrum du muscle. La DMD est une myopathie génétique conduisant à une dégradation progressive de la force musculaire associée à une néovascularisation compensatrice. Le volet clinique/histologique incluant une analyse multiparamétrique des critères évolutifs cliniques et de réponse thérapeutique couplée à une réévaluation des données histologiques de la DMJ (analyse morphométrique des muscles DMJ) a permis de montrer qu’il existait des sous groupes phénotypiques homogènes de sévérité différente dans la DMJ. Le degré de sévérité clinique est relié à la gravité de la vasculopathie musculaire Par ailleurs, des marqueurs cliniques et histologiques simples permettant de repérer au diagnostic les patients nécessitant une escalade thérapeutique rapide (CMAS>34, atteinte gastrointestinale, fibrose endomysiale musculaire au diagnostic) ont été identifiés. Le volet cellulaire a permis l’identification in vitro des interactions cellulaires spécifiques et différentielles des myoblastes issues de patients DMD et DMJ sur les cellules endothéliales normales par l’analyse de leur rôle sur la prolifération, migration et différenciation des cellules vasculaires. Dans la DMD, les myoblastes entrainent une réponse angiogénique importante mais non efficace (néovascularisation anarchique). Dans la DMJ, les myoblastes participent efficacement à la reconstruction vasculaire notamment via la sécrétion de facteurs proangiogéniques. Ces résultats ont été renforcés par analyse transcriptomique effectuée à partir de cellules endothéliales et satellites isolées de muscles de patients confirmant le rôle central de la vasculopathie associée à un contexte inflammatoire spécifique lié à l’interféron dans la physiopathologie de la DMJ et montrant dans la DMD une dérégulation de l’homéostasie normale des interactions vaisseau-muscle avec mise en jeu d’un remodelage tissulaire non efficace. Ces données permettent d'identifier de nouvelles fonctions des cellules vasculaires dans le remodelage du muscle strié squelettique au cours des pathologies musculaires de l'enfant, et devraient ouvrir la voie à de nouvelles approches thérapeutiques. / Skeletal muscle is highly vascularised. Beyond oxygen and nutriment supply, new functions for vessels have been recently identified, through the interactions that vessel cells (endothelial cells) establish with muscle cells, particularly with muscle stem cells (satellite cells). These latter closely interact with endothelial cells for their expansion and their differentiation, then with periendothelial cells for their self-renewal and return to quiescence. During skeletal muscle regeneration endothelial cells reciprocally interact with myogenic cells by direct contact or by releasing soluble factors to promote both myogenesis and angiogenesis processes. Skeletal muscle regeneration typically occurs as a result of a trauma or disease, such as congenital or myopathies. To better understand the role of vessel plasticity in tissue remodeling, we took advantage of two muscular disorders that could be considered as paradigmatic situations of regenerating skeletal muscle in the child: Juvenile Dermatomyositis (JDM), the most frequent inflammatory myopathy and Duchenne Muscular Dystrophy (DMD), the most common type of muscular dystrophy. Although these two muscular disorders share, at the tissue level, similar mechanisms of necrosis-inflammation, they differ regarding the vessel domain. In JDM patients, microvascular changes consist in a destruction of endothelial cells assessed by focal capillary loss. This capillary bed destruction is transient. The tissue remodeling is efficient and muscle may progressively recover its function. By contrast, in DMD, despite an increase of vessels density in an attempt to improve the muscle perfusion, the muscle function progressively alters with age. We identified clinical and pathological markers of severity and predictive factors for poor clinical outcome in JDM by computing a comprehensive initial and follow-up clinical data set with deltoid muscle biopsy alterations controlled by age-based analysis of the deltoid muscle capillarization. We demonstrated that JDM can be divided into two distinctive clinical subgroups. The severe clinical presentation and outcome are linked to vasculopathy. Furthermore, a set of simple predictors (CMAS<34, gastrointestinal involvement, muscle endomysial fibrosis at disease onset) allow early recognition of patients needing rapid therapeutic escalation with more potent drugs. We studied in vitro the specific cell interactions between myogenic cells issued from JDM and DMD patients and normal endothelial cells to explore whether myogenic cells participate to the vessel remodeling observed in the two pathologies. We demonstrated that MPCs possessed angiogenic properties depending on the pathological environment. In DMD, MPCs promoted the development of establishment of an anarchic, although strong, EC stimulation, leading to the formation of weakly functional vessels. In JDM, MPCs enhanced the vessel reconstruction via the secretion of proangiogenic factors. This functional analysis was supported by the transcriptomic analysis consistent with a central vasculopathy in JDM including a strong and specific response to an inflammatory environment. On the contrary, DMD cells presented an unbalanced homeostasis with deregulation of several processes including muscle and vessel development with attempts to recover neuromuscular system by MPCs. To summarize, our data should allow the definition of new functions of vessel cells in skeletal muscle remodelling during muscle pathologies of the child that will open the way to explore new therapeutic options and to gain further insights in the pathogenesis of these diseases.