Two-thirds were to be pikemen and the rest would be armed with halberds , crossbows and arquebuses. The Francs-archers were primarily used for frontier defense against foreign enemies and against bandits in the interior such as the aventuriers.
In addition, they were prone to desertion, treason and capitulation. During the reign of Louis XI the francs-archers performed poorly at the battle of Guinegate while under the command of Marshal Joachim Rouault. Lacking any unit training or discipline, they lost 6, men killed in action and many of them looted the enemy camp instead of fighting.
The franc-archers was a stock figure of fun in literary satire as early as the late 15th century. In these satires the franc-archer is portrayed as vainglorious, cowardly and militarily useless. Cambridge University Press. From Wikipedia, the free encyclopedia. Namespaces Article Talk. Maternal transmission of the deletions was also confirmed in a second fetus who was aborted due to the causality of the deletions.
The genomic positions of these previously described deletions did not overlap with ours. These patients presented ID and short seizures. Thus, to our knowledge, this is the first case of two deletions in the same chromosomal region Xp Severe ID is one of the major sign that presented our patient. In , Hagens et al. They also identified a missense exchange in this gene, segregating with the Stocco dos Santos XLMR syndrome in a large four-generation pedigree but absent in more than control X-chromosomes.
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Among other phenotypic characteristics, the affected males in this family presented with severe ID, delayed or no speech, seizures and hyperactivity. Yoder and Hildebrand described the characterization of mouse SHROOM4 and observed that the protein localizes to a distinct population of F-actin and appears to regulate the formation of this cytoskeletal compartment [ 16 ].
Myosin II is also localized to this compartment and appears to be required for maintaining its proper organization.
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Thus, SHROOM4 encodes a protein involved in cytoskeletal architecture and may function to regulate cellular and cytoskeletal architecture by modulating the spatial distribution of myosin II [ 16 ]. SHROOM4 is also expressed in human adult and fetal brain structures and is needed to establish or maintain a particular cytoskeletal structure that is necessary for the function or survival of a specific neuronal population of cells [ 16 ].
The total loss of function could be responsible for his phenotype. This could include cellular properties such as morphology, vesicular trafficking or polarity, all of which are regulated by the actin cytoskeleton. Based on these observations, it will be important to determine what other developmental processes might be regulated by SHROOM4 and if there are genetic interactions between SHROOM4 and other regulators of cellular morphology.
Others genes could be potentially candidate for the ID of the patient.
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Indeed, western blot analysis of MAGED1 detected expression in embryos, especially in cerebral cortex and cerebellum. MAGED1 plays important roles in the central nervous system in both developmental and adult stages [ 17 ]. CLCN5 chloride channel 5 is located on chromosome Xp Mutations in CLCN5 have been found in Dent disease, an X-linked inherited renal condition characterized by proteinuria, hypercalciuria and hyperphosphaturia, kidney stones, and in some cases, kidney failure [ 11 - 13 ].
To date, mutations in the gene are described and alternatively spliced transcript variants encoding different isoforms have been found. The total number of reported CLCN5 mutations is , and these are scattered throughout the coding region, with no evidence for major mutational hot spots [ 20 ]. The possibility that these other genes may encode some of the proteins e.
Besides the gene content of the two interstitial deletions and the most plausible candidates, one may not exclude position effects on neighboring genes which are not included in the deletions. To our knowledge, this patient is the first description of a double Xp The diagnosis of Dent disease is usefully based on the presence of all three of the following criteria: low-molecular-weight proteinuria, hypercalciuria and at least one of the following: nephrocalcinosis, kidney stones, hematuria, hypophosphatemia or renal insufficiency.
Generally, molecular genetic testing and the presence of a CLCN5 mutation confirm the diagnosis. For our patient, the Xp The genotype-phenotype correlation was discussed with the family during genetic counseling. Prenatal diagnosis was proposed for the second pregnancy and the couple decided, according to the French law, to terminate the pregnancy with an affected male. All findings strongly implicate the inherited Xp The Xp The patient was the product of the first pregnancy of healthy and non-consanguineous parents.
During this first pregnancy the first trimester ultrasound showed an increased nuchal translucency 3. The neonate was found to be mildly hypotonic. Peripheral infusion was inserted for three days until the infant was able to be bottle fed. Walking was acquired at 4.
At the age of four-year-old, this patient was referred for evaluation of severe growth retardation associated with both motor and cognitive delays. Moreover, the child presented renal proximal tubulopathy with low-molecular-weight proteinuria, hypercalciuria, hyperaminoaciduria, hypophosphatemia and hyperuricemia, as observed in Dent disease. Note prominent philtrum, large forehead, retrognathia, thin upper lip and low-set ears. After fetal cytogenetic analysis and according to the French law, the parents decided to terminate the pregnancy.
Parents showed a normal phenotype and no particular personal history. Standard and high resolution chromosome analyses were performed from trophoblast cells and cultured peripheral lymphocytes of the patient using standard procedures G-band by trypsin using Giemsa GTG , R-band after heat denaturation and Giemsa RHG banding techniques and Giemsa staining.
Fluorescent in sit u hybridization FISH analyses were performed on lymphocyte metaphase spreads of the patient, his parents and his maternal grandmother. Bacterial artificial chromosome BAC clones specific for the Xp Captured images were processed with Feature Extraction software The ADM2 algorithm was used for statistical analysis.
Experimental research on patients was done according to French law R. Written informed consent was obtained from the patient's parents for the publication of this report and any accompanying images. The authors thank the family for agreeing to participate in this study.
They thank also all the technical team for array-CGH experiments.
Philippe Labrune and Lucie Tosca contributed equally to this work. Competing interests. NA conceived the study and drafted the manuscript. CM contributed to conception of the study, analysis and interpretation of data. SB participated in the design of the study.
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