RESUMEN
BACKGROUND: Various animal models mimicking craniosynostosis have been developed, using mutant zebrafish and mouse. The aim of this paper is to review the different animal models for syndromic craniosynostosis and analyze what insights they have provided in our understanding of the pathophysiology of these conditions. MATERIAL AND METHODS: The relevant literature for animal models of craniosynostosis was reviewed. RESULTS: Although few studies on craniosynostosis using zebrafish were published, this model appears useful in studying the suture formation mechanisms conserved across vertebrates. Conversely, several mouse models have been generated for the most common syndromic craniosynostoses, associated with mutations in FGFR1, FGFR2, FGFR3 and TWIST genes and also in MSX2, EFFNA, GLI3, FREM1, FGF3/4 genes. The mouse models have also been used to test pharmacological treatments to restore craniofacial growth. CONCLUSIONS: Several zebrafish and mouse models have been developed in recent decades. These animal models have been helpful for our understanding of normal and pathological craniofacial growth. Mouse models mimicking craniosynostoses can be easily used for the screening of drugs as therapeutic candidates.
Asunto(s)
Craneosinostosis/patología , Modelos Animales de Enfermedad , Animales , Craneosinostosis/genética , Humanos , MutaciónRESUMEN
Energy levels, radiative decay, and Auger rates have been calculated for dielectronic recombination of seven Ne-like ions, ranging from Fel7+ to Ag38+, from the n = 2 F-like shell into the n = 3 one, by use of the multiconfiguration AUTOLSJ code in intermediate coupling. Rate coefficients have then been computed versus the electronic temperature, the nuclear charge of the involved element, the final Ne-like state, and the population distribution among the initial 3 F-like levels. Dielectronic spectra have also been obtained.
RESUMEN
Amnesic patients and control subjects were asked to reproduce arm movements of a given amplitude and to average amplitudes on series of three such movements. The error in averaging was found to be equal in patients and control subjects while reproduction performance was clearly affected by amnesia. The implications of these data for a theory of amnesia are briefly discussed.
