about
Secretory cytotoxic granule maturation and exocytosis require the effector protein hMunc13-4Griscelli syndrome restricted to hypopigmentation results from a melanophilin defect (GS3) or a MYO5A F-exon deletion (GS1)Munc18-2 deficiency causes familial hemophagocytic lymphohistiocytosis type 5 and impairs cytotoxic granule exocytosis in patient NK cellsA newly identified isoform of Slp2a associates with Rab27a in cytotoxic T cells and participates to cytotoxic granule secretionRIAM links the ADAP/SKAP-55 signaling module to Rap1, facilitating T-cell-receptor-mediated integrin activationMutations in RAB27A cause Griscelli syndrome associated with haemophagocytic syndromeHDAC6 regulates the dynamics of lytic granules in cytotoxic T lymphocytes.EBAG9 tempers lymphocyte killing activity.Primary hemophagocytic syndromes point to a direct link between lymphocyte cytotoxicity and homeostasis.Rab27A and its effector MyRIP link secretory granules to F-actin and control their motion towards release sitesRegulation of T-cell antigen receptor-mediated inside-out signaling by cytosolic adapter proteins and Rap1 effector molecules.Kinesin-1 controls mast cell degranulation and anaphylaxis through PI3K-dependent recruitment to the granular Slp3/Rab27b complex.Inherited defects in lymphocyte cytotoxic activity.A RAB27A duplication in several cases of Griscelli syndrome type 2: An explanation for cases lacking a genetic diagnosis.Severe and progressive encephalitis as a presenting manifestation of a novel missense perforin mutation and impaired cytolytic activity.Polygenic mutations in the cytotoxicity pathway increase susceptibility to develop HLH immunopathology in mice.A novel immunoregulatory role for NK-cell cytotoxicity in protection from HLH-like immunopathology in mice.Griscelli syndrome types 1 and 2.Terminal transport of lytic granules to the immune synapse is mediated by the kinesin-1/Slp3/Rab27a complex.Griscelli disease: genotype-phenotype correlation in an array of clinical heterogeneity.Griscelli syndrome restricted to hypopigmentation results from a melanophilin defect (GS3) or a MYO5A F-exon deletion (GS1).Griscelli syndrome: characterization of a new mutation and rescue of T-cytotoxic activity by retroviral transfer of RAB27A gene.Kinesin-1 Is a New Actor Involved in Platelet Secretion and Thrombus Stability.Inherited defects causing hemophagocytic lymphohistiocytic syndromeLYST Controls the Biogenesis of the Endosomal Compartment Required for Secretory Lysosome Function[Defect in lytic granule exocytosis: several causes, a same effect]Premature birth, respiratory distress, intracerebral hemorrhage, and silvery-gray hair: differential diagnosis of the 3 types of Griscelli syndromeDistinct severity of HLH in both human and murine mutants with complete loss of cytotoxic effector PRF1, RAB27A, and STX11Tomosyn functions as a PKCδ-regulated fusion clamp in mast cell degranulationKinesin-1 regulates antigen cross-presentation through the scission of tubulations from early endosomes in dendritic cells
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description
hulumtues
@sq
researcher
@en
wetenschapper
@nl
հետազոտող
@hy
name
Gael Ménasche
@ast
Gael Ménasche
@en
Gael Ménasche
@es
Gael Ménasche
@nl
Gael Ménasche
@sl
type
label
Gael Ménasche
@ast
Gael Ménasche
@en
Gael Ménasche
@es
Gael Ménasche
@nl
Gael Ménasche
@sl
prefLabel
Gael Ménasche
@ast
Gael Ménasche
@en
Gael Ménasche
@es
Gael Ménasche
@nl
Gael Ménasche
@sl
P106
P21
P31
P496
0000-0001-6671-3642