Search results for "PRR"
showing 10 items of 26 documents
The grapevine flagellin receptor VvFLS2 differentially recognizes flagellin-derived epitopes from the endophytic growth-promoting bacterium Burkholde…
2014
International audience; The role of flagellin perception in the context of plant beneficial bacteria still remains unclear. Here, we characterized the flagellin sensing system flg22-FLAGELLIN SENSING 2 (FLS2) in grapevine, and analyzed the flagellin perception in the interaction with the endophytic plant growth-promoting rhizobacterium (PGPR) Burkholderia phytofirmans. The functionality of the grapevine FLS2 receptor, VvFLS2, was demonstrated by complementation assays in the Arabidopsis thaliana fls2 mutant, which restored flg22-induced H2O2 production and growth inhibition. Using synthetic flg22 peptides from different bacterial origins, we compared recognition specificities between VvFLS2…
Benign nocturnal alternating hemiplegia of childhood
2018
Objective: To describe the clinical spectrum of benign nocturnal alternating hemiplegia of childhood (BNAHC) including long-term follow-up data of previously published cases and to propose an underlying genetic cause of this disorder. Methods: We studied the medical data of two novel patients, reviewed the literature on BNAHC, and gathered information of the most recent follow-up of published cases regarding the course of episodes, further development, attempted drugs, ancillary investigations, and sequelae. Results: All patients, i.e. two novel cases and twelve patients identified in the literature (13 boys, 1 girl, age at onset four months to three years), experienced episodes of hemipleg…
PRRT2 gene variant in a child with dysmorphic features, congenital microcephaly, and severe epileptic seizures: genotype-phenotype correlation?
2019
Abstract Background Mutations in Proline-rich Transmembrane Protein 2 (PRRT2) have been primarily associated with individuals presenting with infantile epilepsy, including benign familial infantile epilepsy, benign infantile epilepsy, and benign myoclonus of early infancy, and/or with dyskinetic paroxysms such as paroxysmal kinesigenic dyskinesia, paroxysmal non-kinesigenic dyskinesia, and exercise-induced dyskinesia. However, the clinical manifestations of this disorder vary widely. PRRT2 encodes a protein expressed in the central nervous system that is mainly localized in the pre-synaptic neurons and is involved in the modulation of synaptic neurotransmitter release. The anomalous functio…
Lutter contre les infections bactériennes : le système immunitaire des plantes est aussi très efficace !
2016
SPE IPM UB CNRS Agrosup
PRRT2 mutations are the major cause of benign familial infantile seizures.
2012
Mutations in PRRT2 have been described in paroxysmal kinesigenic dyskinesia (PKD) and infantile convulsions with choreoathetosis (PKD with infantile seizures), and recently also in some families with benign familial infantile seizures (BFIS) alone. We analyzed PRRT2 in 49 families and three sporadic cases with BFIS only of Italian, German, Turkish, and Japanese origin and identified the previously described mutation c.649dupC in an unstable series of nine cytosines to occur in 39 of our families and one sporadic case (77% of index cases). Furthermore, three novel mutations were found in three other families, whereas 17% of our index cases did not show PRRT2 mutations, including a large fami…
L’autonomia di spesa delle Regioni fra Stato ed Unione europea dalla l. cost. 3/2001 alle condizionalità europee sulla ripresa e la resilienza
2021
L'articolo ricostruisce la tendenza all’accentramento nel governo delle finanze pubbliche e l’eccessiva distanza fra testo costituzionale e diritto costituzionale vivente per analizzare l'attuazione del Recovery Plan (PNRR) rilevando un ulteriore tendenza all’accentramento del governo della spesa. Si indagano dunque le ragioni della compressione dell’autonomia finanziaria di spesa per effetto del principio di indifferenza e del “doppio intreccio federale”. Si mira a inquadrare il tema alla luce della leale collaborazione e dellala resilienza istituzionale per accorciare la “distanza” fra testo costituzionale ed attuazione costituzionale in tema di autonomia di spesa delle Regioni. Si prospe…
Identification of the cell-wall derived xyloglucan as a new damage-associated molecular pattern (DAMP) eliciting plant immunity in Vitis vinifera and…
2018
Activation of the plant immune responses requires recognition of common pathogen-associated molecular pattern (PAMP) by their cognate pattern recognition receptors (PRR). Chitin, a major component of fungal cell walls, is a well-known PAMP that triggers defense responses in several mammal and plant species.In the first part of this study, we show that two chitooligosaccharides, chitin and chitosan, act as PAMPs in grapevine (Vitis vinifera) as they elicit immune signaling events, defense gene expression, and resistance against pathogens. These two PAMPs are active in grapevine suggesting that at least one perception system exists. Phylogenetic analysis clearly distinguished three V. vinifer…
Mucosal immunology and probiotics.
2013
The cross-talk between the mucosa-associated immune system and microbiota is critical in mucosal tissue homeostasis as well as in protection against infectious and inflammatory diseases occurring at mucosal sites. This recent evidence has paved the way to therapeutic approaches aimed at modulating the mucosa-associated immune system using probiotics. Different strains of probiotics possess the ability to finely regulate dendritic cell (DC) activation, polarizing the subsequent T cell activity toward Th1 (e.g. Lactobacillus (Lb) acidophilus), Th2 (Lb.reuteri and Bifidobacterium bifidum) or, as more recently demonstrated, Th17 responses induced by specific strains such as Lb.rhamnosus GG and …
Autonomie e controlli della Corte dei conti nel quadro dei principi costituzionali e dei vincoli sovranazionali in materia economico-finanziaria
2022
Una riflessione dei controlli sulle autonomie territoriali intestati alla Corte dei conti non può prescindere dal loro inquadramento costituzionale. L’A. prospetta una ricostruzione del controllo come espressione di una funzione neutra e imparziale che deve riposare su precisi parametri costituzionali che ne legittimano l’adozione e di cui va resa una stretta interpretazione: il controllo sulle Autonomie territoriali deve così essere necessariamente una attività tale da avvicinarla a quella giurisdizionale piuttosto che amministrativa. È in tale chiave che deve essere inteso il rapporto fra ausiliarietà di cui all’art. 100 Cost. e controlli sui bilanci ed i rendiconti. L’evoluzione del quad…
Updated insights into the mechanism of action and clinical profile of the immunoadjuvant QS-21: A review
2019
Background Vaccine adjuvants are compounds that significantly enhance/prolong the immune response to a co-administered antigen. The limitations of the use of aluminium salts that are unable to elicite cell responses against intracellular pathogens such as those causing malaria, tuberculosis, or AIDS, have driven the development of new alternative adjuvants such as QS-21, a triterpene saponin purified from Quillaja saponaria. Purpose The aim of this review is to attempt to clarify the mechanism of action of QS-21 through either receptors or signaling pathways in vitro and in vivo with special emphasis on the co-administration with other immunostimulants in new adjuvant formulations, called a…