1,720,977 research outputs found
Functional Interaction Of Common Gamma Chain And Growth Hormone Receptor Signaling Apparatus.
No full textEvaluation of functional interaction between the common gamma chain and growth hormone receptor using a neutralization assay.
No full textGH-induced signaling and STAT5b nuclear translocation in control or X-SCID EBV cell lines.
No full textTransduction of ?-X-SCID EBV cells with type? C gene restores GH-induced STAT5 phosphorylation.
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Functional interaction of common gamma-chain and growth hormone receptor signaling apparatus.
No full textFOXN1 homozygous mutation associated with anencephaly and severe neural tube defect in human athymic Nude/SCID fetus
No full textSignificant expansion of TCR gamma/delta+ CD4 and CD8 double negative (DN) T cells and aftous stomatitis resistant to therapy: clinical report.
No full textFunctional interaction of common gamma-chain and growth hormone receptor signaling apparatus
No full textWe previously reported on an X-linked SCID (X-SCID) patient, who also had peripheral growth hormone (GH) hyporesponsiveness and abnormalities of the protein phosphorylation events following GH receptor (GHR) stimulation. In the present study, we examined a potential role of common cytokine receptor gamma-chain (gammac) in GHR signaling using EBV-transformed lymphocytes from healthy subjects and gammac-negative X-SCID patients. We demonstrated that the proliferative response to GH stimulation of the B cell lines of gammac-negative patients was impaired despite a comparable cellular expression of GHR molecules to controls. In patients, after GH stimulation, no phosphorylation of STAT5 was observed. In addition, the molecule localization through confocal microscopy revealed that in B cell lines of patients no nuclear translocation of STAT5b following GH stimulation occurred differently from controls. Biochemical analysis of the nuclear extracts of gammac-negative cell lines provided further evidence that the amount of STAT5b and its phosphorylated form did not increase following GH stimulation. In patients, cells reconstituted with wild-type gammac abnormal biochemical and functional events were restored resulting in nuclear translocation of STAT5. Confocal experiments revealed that GHR and gammac were colocalized on the cell membrane. Our study demonstrates the existence of a previously unappreciated relationship between GHR-signaling pathway and gammac, which is required for the activation of STAT5b in B cell lines. These data also confirm that growth failure in X-SCID is primarily related to the genetic alteration of the IL2RG gene
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