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Rac2 Deficiency

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  • First Online:
Encyclopedia of Medical Immunology

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Synonyms

Neutrophil immunodeficiency syndrome

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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Author information

Authors and Affiliations

  1. Pediatric Department, Allergy and the Immunology Services, “Edmond and Lily Safra” Children’s Hospital, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel

    Raz Somech & Tali Stauber

  2. Jeffrey Modell Foundation Center, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel

    Raz Somech & Tali Stauber

  3. Sheba Medical Center, Tel Hashomer, Sackler School of Medicine, Tel Aviv University, Tel Aviv, Israel

    Raz Somech & Tali Stauber

Authors
  1. Raz Somech
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  2. Tali Stauber
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Corresponding author

Correspondence to Raz Somech .

Editor information

Editors and Affiliations

  1. Molecular Biology, Monash University Dept. Biochemistry &, Clayton, Victoria, Australia

    Ian MacKay

  2. Autoimmune Disease Research Center, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA

    Noel R. Rose

Section Editor information

  1. Institute of Biomedical Sciences, University of São Paulo, São Paulo, São Paulo, Brasil

    Antonio Condino-Neto

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Somech, R., Stauber, T. (2018). Rac2 Deficiency. In: MacKay, I., Rose, N. (eds) Encyclopedia of Medical Immunology. Springer, New York, NY. https://doi.org/10.1007/978-1-4614-9209-2_142-1

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  • DOI: https://doi.org/10.1007/978-1-4614-9209-2_142-1

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-9209-2

  • Online ISBN: 978-1-4614-9209-2

  • eBook Packages: Springer Reference MedicineReference Module Medicine

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