Encyclopedia of Medical Immunology

Living Edition
| Editors: Ian MacKay, Noel R. Rose

Rac2 Deficiency

  • Raz SomechEmail author
  • Tali Stauber
Living reference work entry
DOI: https://doi.org/10.1007/978-1-4614-9209-2_142-1

Synonyms

Definition

Rac2 which is expressed in cells of hematopoietic origin is a regulatory element that constitutes the predominant NADPH oxidase complex in leukocytes. Thus, it regulates superoxide production, direct migration in neutrophils and play distinct roles in actin organization, cell survival, and proliferation. Its deficiency leads to an immunodeficiency clinically similar to Chronic Granulomatous Diseases. Interestingly, it was shown that RAC2 is involved also in T and B cell immunity.

Introduction

Rho GTPases, members of the Ras superfamily of GTPases, regulate a wide variety of cellular activities including actin cytoskeleton reorganization, chemotaxis, gene transcription, cell growth, and development. These proteins are divided into six families based on sequence homology, protein domains, and function. Of them, Rac (Ras-related C3 botulinum toxin) proteins composed of three different isoforms, namely, Rac1, Rac2, and Rac3. Rac1 and...

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References

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Copyright information

© Springer Science+Business Media LLC 2018

Authors and Affiliations

  1. 1.Pediatric Department, Allergy and the Immunology Services, “Edmond and Lily Safra” Children’s HospitalSackler School of Medicine, Tel Aviv UniversityTel AvivIsrael
  2. 2.Jeffrey Modell Foundation CenterSackler School of Medicine, Tel Aviv UniversityTel AvivIsrael
  3. 3.Sheba Medical Center, Tel HashomerSackler School of Medicine, Tel Aviv UniversityTel AvivIsrael

Section editors and affiliations

  • Antonio Condino-Neto
    • 1
  1. 1.Institute of Biomedical SciencesUniversity of São PauloSão PauloBrasil