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Brain migration disorder and T-cell activation deficiency associated with abnormal signaling through TCR/CD3 complex and hyperactivity of Fyn tyrosine kinase.

Publication ,  Journal Article
Del Giudice, E; Gaetaniello, L; Matrecano, E; Cosentini, E; Ursini, MV; Racioppi, L; Arrigo, G; Pignata, C
Published in: Neuropediatrics
October 2000

In this study we report on a patient affected by a brain migration disorder and a T-cell activation deficiency presumably inherited as an autosomal recessive trait. The immunological evaluation revealed that the mitogen stimulation failed to induce a proper up-regulation of membrane expression of T-cell activation markers, and cell proliferation. This functional impairment was associated with abnormalities of the signal transduction process that follows T-cell receptor stimulation. A constitutive hyperphosphorylation of the Fyn tyrosine kinase was documented. This is the first report on a T-cell signaling abnormality associated with a developmental brain disorder. Whether the alteration of Fyn, which plays a role in both neurological and immunological systems, is responsible for either disorder remains to be elucidated.

Duke Scholars

Published In

Neuropediatrics

DOI

ISSN

0174-304X

Publication Date

October 2000

Volume

31

Issue

5

Start / End Page

265 / 268

Location

Germany

Related Subject Headings

  • Signal Transduction
  • Receptors, Antigen, T-Cell
  • Proto-Oncogene Proteins c-fyn
  • Proto-Oncogene Proteins
  • Protein-Tyrosine Kinases
  • Phosphorylation
  • Neurology & Neurosurgery
  • Male
  • Immunologic Deficiency Syndromes
  • Humans
 

Citation

APA
Chicago
ICMJE
MLA
NLM
Del Giudice, E., Gaetaniello, L., Matrecano, E., Cosentini, E., Ursini, M. V., Racioppi, L., … Pignata, C. (2000). Brain migration disorder and T-cell activation deficiency associated with abnormal signaling through TCR/CD3 complex and hyperactivity of Fyn tyrosine kinase. Neuropediatrics, 31(5), 265–268. https://doi.org/10.1055/s-2000-9234
Del Giudice, E., L. Gaetaniello, E. Matrecano, E. Cosentini, M. V. Ursini, L. Racioppi, G. Arrigo, and C. Pignata. “Brain migration disorder and T-cell activation deficiency associated with abnormal signaling through TCR/CD3 complex and hyperactivity of Fyn tyrosine kinase.Neuropediatrics 31, no. 5 (October 2000): 265–68. https://doi.org/10.1055/s-2000-9234.
Del Giudice E, Gaetaniello L, Matrecano E, Cosentini E, Ursini MV, Racioppi L, et al. Brain migration disorder and T-cell activation deficiency associated with abnormal signaling through TCR/CD3 complex and hyperactivity of Fyn tyrosine kinase. Neuropediatrics. 2000 Oct;31(5):265–8.
Del Giudice, E., et al. “Brain migration disorder and T-cell activation deficiency associated with abnormal signaling through TCR/CD3 complex and hyperactivity of Fyn tyrosine kinase.Neuropediatrics, vol. 31, no. 5, Oct. 2000, pp. 265–68. Pubmed, doi:10.1055/s-2000-9234.
Del Giudice E, Gaetaniello L, Matrecano E, Cosentini E, Ursini MV, Racioppi L, Arrigo G, Pignata C. Brain migration disorder and T-cell activation deficiency associated with abnormal signaling through TCR/CD3 complex and hyperactivity of Fyn tyrosine kinase. Neuropediatrics. 2000 Oct;31(5):265–268.
Journal cover image

Published In

Neuropediatrics

DOI

ISSN

0174-304X

Publication Date

October 2000

Volume

31

Issue

5

Start / End Page

265 / 268

Location

Germany

Related Subject Headings

  • Signal Transduction
  • Receptors, Antigen, T-Cell
  • Proto-Oncogene Proteins c-fyn
  • Proto-Oncogene Proteins
  • Protein-Tyrosine Kinases
  • Phosphorylation
  • Neurology & Neurosurgery
  • Male
  • Immunologic Deficiency Syndromes
  • Humans