794 research outputs found

    La SPIR, un outil pour l'alimentation de précision, de l'usine à l'élevage

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    La spectrométrie dans le proche infrarouge (SPIR) est une technique analytique qui a connu un développement important dans le domaine de l'alimentation animale. Rapide et peu coûteuse, elle trouve sa place dans un contexte de besoin croissant de contrôle qualité et de traçabilité. La première utilisation de la SPIR est l'analyse des matières premières, depuis leur production (organismes stockeurs) jusqu'à la réception des lots chez les fabricants d'aliment. La SPIR permet de mesurer la composition chimique (humidité, protéines, lipides, etc.) mais également d'estimer la valeur nutritive (digestibilité des acides aminés, énergie métabolisable) et se révèle donc un outil précieux pour la formulation. Au cours de la fabrication comme sur les produits finis, la SPIR permet aussi d'analyser les aliments composés et de vérifier leur conformité aux formules théoriques. Les nouveaux matériels permettent de mettre en place des mesures directement " en ligne " lors de la production. L'utilisation de la SPIR peut dépasser l'usine, et des résultats récents montrent que des perspectives intéressantes se dessinent pour des mesures de digestibilité beaucoup plus proches du terrain que les expérimentations classiques. Enfin la caractérisation des effluents permet à la fois de mieux orienter leur utilisation et se servir de l'information (humidité, azote) comme aide au diagnostic de problèmes d'élevage. (Résumé d'auteur

    An Extended Network of Genomic Maintenance in the Archaeon Pyrococcus abyssi Highlights Unexpected Associations between Eucaryotic Homologs.

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    In Archaea, the proteins involved in the genetic information processing pathways, including DNA replication, transcription, and translation, share strong similarities with those of eukaryotes. Characterizations of components of the eukaryotic-type replication machinery complex provided many interesting insights into DNA replication in both domains. In contrast, DNA repair processes of hyperthermophilic archaea are less well understood and very little is known about the intertwining between DNA synthesis, repair and recombination pathways. The development of genetic system in hyperthermophilic archaea is still at a modest stage hampering the use of complementary approaches of reverse genetics and biochemistry to elucidate the function of new candidate DNA repair gene. To gain insights into genomic maintenance processes in hyperthermophilic archaea, a protein-interaction network centred on informational processes of Pyrococcus abyssi was generated by affinity purification coupled with mass spectrometry. The network consists of 132 interactions linking 87 proteins. These interactions give insights into the connections of DNA replication with recombination and repair, leading to the discovery of new archaeal components and of associations between eucaryotic homologs. Although this approach did not allow us to clearly delineate new DNA pathways, it provided numerous clues towards the function of new molecular complexes with the potential to better understand genomic maintenance processes in hyperthermophilic archaea. Among others, we found new potential partners of the replication clamp and demonstrated that the single strand DNA binding protein, Replication Protein A, enhances the transcription rate, in vitro, of RNA polymerase. This interaction map provides a valuable tool to explore new aspects of genome integrity in Archaea and also potentially in Eucaryotes

    Circulating levels of dickkopf-1, osteoprotegerin and sclerostin are higher in old compared with young men and women and positively associated with whole-body bone mineral density in older adults

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    Summary: Bone mineral density declines with increasing older age. We examined the levels of circulating factors known to regulate bone metabolism in healthy young and older adults. The circulating levels of dickkopf-1, osteocalcin, osteoprotegerin and sclerostin were positively associated with WBMD in older adults, despite the average WBMD being lower and circulating dickkopf-1, osteoprotegerin and sclerostin being higher in old than young. Purpose: To investigate the relationship between whole-body bone mineral density (WBMD) and levels of circulating factors with known roles in bone remodelling during 'healthy' ageing. Methods: WBMD and fasting plasma concentrations of dickkopf-1, fibroblast growth factor-23, osteocalcin, osteoprotegerin, osteopontin and sclerostin were measured in 272 older subjects (69 to 81 years; 52% female) and 171 younger subjects (18-30 years; 53% female). Results: WBMD was lower in old than young. Circulating osteocalcin was lower in old compared with young, while dickkopf-1, osteoprotegerin and sclerostin were higher in old compared with young. These circulating factors were each positively associated with WBMD in the older adults and the relationships remained after adjustment for covariates (r-values ranging from 0.174 to 0.254, all p<0.01). In multivariate regression, the body mass index, circulating sclerostin and whole-body lean mass together accounted for 13.8% of the variation with WBMD in the older adults. In young adults, dickkopf-1 and body mass index together accounted for 7.7% of variation in WBMD. Conclusion: Circulating levels of dickkopf-1, osteocalcin, osteoprotegerin and sclerostin are positively associated with WBMD in community-dwelling older adults, despite the average WBMD being lower and circulating dickkopf-1, osteoprotegerin and sclerostin being higher in old than young

    Gait characterization in golden retriever muscular dystrophy dogs using linear discriminant analysis

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    Background: Accelerometric analysis of gait abnormalities in golden retriever muscular dystrophy (GRMD) dogs isof limited sensitivity, and produces highly complex data. The use of discriminant analysis may enable simpler andmore sensitive evaluation of treatment benefits in this important preclinical model.Methods: Accelerometry was performed twice monthly between the ages of 2 and 12 months on 8 healthy and20 GRMD dogs. Seven accelerometric parameters were analysed using linear discriminant analysis (LDA). Manipulationof the dependent and independent variables produced three distinct models. The ability of each model to detect gaitalterations and their pattern change with age was tested using a leave-one-out cross-validation approach.Results: Selecting genotype (healthy or GRMD) as the dependent variable resulted in a model (Model 1) allowing agood discrimination between the gait phenotype of GRMD and healthy dogs. However, this model was not sufficientlyrepresentative of the disease progression. In Model 2, age in months was added as a supplementary dependentvariable (GRMD_2 to GRMD_12 and Healthy_2 to Healthy_9.5), resulting in a high overall misclassification rate (83.2%).To improve accuracy, a third model (Model 3) was created in which age was also included as an explanatory variable.This resulted in an overall misclassification rate lower than 12%. Model 3 was evaluated using blinded data pertainingto 81 healthy and GRMD dogs. In all but one case, the model correctly matched gait phenotype to the actualgenotype. Finally, we used Model 3 to reanalyse data from a previous study regarding the effects ofimmunosuppressive treatments on muscular dystrophy in GRMD dogs. Our model identified significant effect ofimmunosuppressive treatments on gait quality, corroborating the original findings, with the added advantages ofdirect statistical analysis with greater sensitivity and more comprehensible data representation.Conclusions: Gait analysis using LDA allows for improved analysis of accelerometry data by applying adecision-making analysis approach to the evaluation of preclinical treatment benefits in GRMD dogs

    Assessment of the upper limb function, strength, and mobility in treatment-naive children with spinal muscular atrophy Types 2 and 3

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    INTRODUCTION/AIMS: Current upper limb assessments in pediatric spinal muscular atrophy (SMA) may not adequately capture change with disease progression. Our aim was to examine the relationship between motor function, strength, and hand/finger mobility of the upper limb in treatment-naïve children with SMA Types 2 and 3 to assess new methods to supplement current outcomes. METHODS: The Revised Upper Limb Module (RULM), grip and pinch strength, and hand/finger mobility data were collected from 19 children with SMA Types 2 and 3 aged 5.2-16.9 years over a year. RESULTS: A median loss between 0.5 and 2.5 points in the RULM was seen across all SMA subgroups with the biggest median loss recorded between 10 and 14 years of age. The grip strength loss was -0.06 kg (-4.69 to 3.49; IQR, 1.21); pinch improvement of 0.05 (-0.65 to 1.27; IQR, 0.48); hand/finger mobility test improvement of 4 points (-24 to 14; IQR, 6.75) for the whole cohort. Significant correlations were found between the RULM and grip strength (p < .001), RULM and pinch strength (p < .001), RULM and revised Brooke (p < .001), grip strength and pinch strength (p < .001). DISCUSSION: The combined use of the RULM, dynamometry, and hand mobility provide insight about correlations between function and strength in children with SMA. The RULM and grip strength assessments captured a significant decline in upper limb function, whereas the pinch and finger/hand mobility showed an improvement over the course of 1 year and these results should be considered for future studies

    Relationship Between Hand Strength and Function in Duchenne Muscular Dystrophy and Spinal Muscular Atrophy: Implications for Clinical Trials.

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    peer reviewed[en] BACKGROUND: Measurement of muscle strength and motor function is recommended in clinical trials of neuromuscular diseases, but the loss of hand strength at which motor function is impacted is not documented. OBJECTIVES: To establish the relationship between hand strength and function, and to determine the strength threshold that differentiates normal and abnormal hand function in individuals with Duchenne Muscular Dystrophy (DMD) or Spinal Muscular Atrophy (SMA). METHODS: Maximal handgrip and key pinch strength were measured with the MyoGrip and MyoPinch dynamometers, respectively. Hand function was assessed using the MoviPlate, the Motor Function Measure items for distal upper limb (MFM-D3-UL) and the Cochin Hand Function Scale (CHFS). RESULTS: Data from 168 participants (91 DMD and 77 SMA, age 6-31 years) were analyzed. Relationships between strength and function were significant (P < 0.001). Hand function was generally preserved when strength was above the strength threshold determined by Receiver-Operating Characteristic (ROC) analysis: For MFM-D3-UL, the calculated handgrip strength thresholds were 41 and 13% of the predicted strength for a healthy subject (% pred) and the key pinch strength thresholds were 42 and 26% pred for DMD and SMA, respectively. For the MoviPlate, handgrip strength thresholds were 11 and 8% pred and key pinch strength thresholds were 21 and 11% pred for DMD and SMA, respectively. For participants with sub-threshold strength, hand function scores decreased with decreasing strength. At equal % pred strength, individuals with SMA had better functional scores than those with DMD. CONCLUSIONS: Hand function is strength-dependent for most motor tasks. It declines only when strength falls below a disease-specific threshold. Therefore, therapies capable of maintaining strength above this threshold should preserve hand function

    Upper limb disease evolution in exon 53 skipping eligible patients with Duchenne muscular dystrophy

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    Objective: To understand the natural disease upper limb progression over 3 years of ambulatory and non-ambulatory patients with Duchenne muscular dystrophy (DMD) using functional assessments and quantitative magnetic resonance imaging (MRI) and to exploratively identify prognostic factors. Methods: Forty boys with DMD (22 non-ambulatory and 18 ambulatory) with deletions in dystrophin that make them eligible for exon 53-skipping therapy were included. Clinical assessments, including Brooke score, motor function measure (MFM), hand grip and key pinch strength, and upper limb distal coordination and endurance (MoviPlate), were performed every 6 months and quantitative MRI of fat fraction (FF) and lean muscle cross sectional area (flexor and extensor muscles) were performed yearly. Results: In the whole population, there were strong nonlinear correlations between outcome measures. In non-ambulatory patients, annual changes over the course of 3 years were detected with high sensitivity standard response mean (|SRM| ≥0.8) for quantitative MRI-based FF, hand grip and key pinch, and MFM. Boys who presented with a FF27% were able to bring a glass to their mouth and retained this ability in the following 3 years. Ambulatory patients with grip strength >35% of predicted value and FF <10% retained ambulation 3 years later. Interpretation: We demonstrate that continuous decline in upper limb strength, function, and MRI measured muscle structure can be reliably measured in ambulatory and non-ambulatory boys with DMD with high SRM and strong correlations between outcomes. Our results suggest that a combination of grip strength and FF can be used to predict important motor milestones

    Dystrophin isoform deficiency and upper-limb and respiratory function in Duchenne muscular dystrophy

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    \ua9 2025 The Author(s). Developmental Medicine &amp; Child Neurology published by John Wiley &amp; Sons Ltd on behalf of Mac Keith Press. Aim: To investigate the associations between mutations expected to differentially affect Dp140 expression and long-term trajectories of respiratory and upper-limb motor outcomes in Duchenne muscular dystrophy (DMD). Method: In a retrospective analysis of population-based longitudinal data from three real-world and natural history data sources, individuals with DMD aged 5 years to 18 years were subdivided according to the predicted effects of the participants\u27 DMD mutation on dystrophin isoform expression (group 1, Dp427 absent, Dp140/Dp71 present; group 2, Dp427/Dp140 absent, Dp71 present). Results: A total of 459 participants were studied, with upper-limb outcomes assessed in 71 (27 in group 1 and 44 in group 2) and forced vital capacity percentage predicted (%pred) assessed in 434 (224 in group 1 and 210 in group 2). Mean grip strength %pred was on average 7.1 percentage points lower in group 2 than in group 1 (p = 0.03). Mean pinch strength %pred was on average 9.2 percentage points lower in group 2 than in group 1 (p = 0.04). Mean forced vital capacity %pred was on average 4.3 percentage points lower in group 2 than in group 1 (p = 0.01). Interpretation: In individuals with DMD, DMD mutations predicted to affect Dp140 expression were associated with more severe trajectories of respiratory and upper-limb motor outcomes

    Determinants of Performance in the Timed Up-and-Go and Six-Minute Walk Tests in Young and Old Healthy Adults

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    The aim of this study was to assess associations between performance in the timed up-and-go (TUG) and six-minute walk distance (6MWD) with physiological characteristics in young and old healthy adults. Thereto, we determined TUG, 6MWD, normalised jump power, centre of pressure displacement during 1-leg standing, forced expiratory volume in 1 s, percentage of age-predicted maximal heart rate (HR%) and height in 419 healthy young (men: 23.5 ± 2.8 years, women: 23.2 ± 2.9 years) and old (men: 74.6 ± 3.2 years, women: 74.1 ± 3.2 years) adults. Normalised jump power explained 8% and 19% of TUG in young (p = 0.025) and older men (p < 0.001), respectively. When fat mass percentage and age were added to normalised jump power, 30% of TUG was explained in older men (R2adj = 0.30, p < 0.001 to 0.106). Appendicular lean muscle mass percentage (ALM%) and age were the best determinants of TUG for older women (R2adj = 0.16, p < 0.001 to 0.01). HR% explained 17–39% of 6MWD across all groups (R2adj = 0.17 to 39, p < 0.001). In conclusion, in men, jump power was a key determinant for TUG, while in old women only it was the ALM%. As HR% was the most important determinant of 6MWD, motivational bias needs to be considered in the interpretation of this test
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