Éditeur(s) : HAL CCSD Springer Verlag/EDP Sciences
Résumé : International audience
Context: Woodcutting is a very old technology but scientific research is scarce on the subject. In the last 50 years much work on basic mechanisms as well as industrial processes has been done in France. Aims: The specific nature of wood introduces strong differences between wood and metal cutting processes. The paper focuses on French teams' contributions. Results: The basic aspects of the tool–material interaction for different basic modes in woodcutting are highlighted. In primary conversion such as sawing, veneer cutting or green wood chipping, huge progress comes from automation and the possibility of linking the process to log and product quality through new sensors. In secondary processing much has been done on the links between the cutting process, surface qualification and the properties of these surfaces for further processing, such as gluing or coating. Tool wear depends on the cutting process, timber quality, and species. Trade-offs are required in tool technology and coating technologies may improve tool life. Conclusion: A large amount of knowledge and innovation has come from 50 years of worldwide research effort, with France being particularly active in this period. The transfer of skills from metals cutting industry was often a key, but much is needed to move closer to both metal cutting sector and woodcutting skills among craftsmen.
ISSN: 1286-4560

hal-01135156
https://hal.archives-ouvertes.fr/hal-01135156
https://hal.archives-ouvertes.fr/hal-01135156/document
https://hal.archives-ouvertes.fr/hal-01135156/file/Wood_machining_focus_French_research_Thibaut_al_2015.pdf
DOI : 10.1007/s13595-015-0460-2

Éditeur(s) : HAL CCSD Nature Publishing Group
Résumé : International audience
Treacher Collins/Franceschetti syndrome (TCS; OMIM 154500) is a disorder of craniofacial development belonging to the heterogeneous group of mandibulofacial dysostoses. TCS is classically characterized by bilateral mandibular and malar hypoplasia, downward-slanting palpebral fissures, and microtia. To date, three genes have been identified in TCS:,TCOF1, POLR1D, and POLR1C. We report a clinical and extensive molecular study, including TCOF1, POLR1D, POLR1C, and EFTUD2 genes, in a series of 146 patients with TCS. Phenotype-genotype correlations were investigated for 19 clinical features, between TCOF1 and POLR1D, and the type of mutation or its localization in the TCOF1 gene. We identified 92/146 patients (63%) with a molecular anomaly within TCOF1, 9/146 (6%) within POLR1D, and none within POLR1C. Among the atypical negative patients (with intellectual disability and/or microcephaly), we identified four patients carrying a mutation in EFTUD2 and two patients with 5q32 deletion encompassing TCOF1 and CAMK2A in particular. Congenital cardiac defects occurred more frequently among patients with TCOF1 mutation (7/92, 8%) than reported in the literature. Even though TCOF1 and POLR1D were associated with extreme clinical variability, we found no phenotype-genotype correlation. In cases with a typical phenotype of TCS, 6/146 (4%) remained with an unidentified molecular defect.
ISSN: 1098-3600

hal-01134364
https://hal-univ-rennes1.archives-ouvertes.fr/hal-01134364
DOI : 10.1038/gim.2015.29
PUBMED : 25790162