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Chronic Mucocutaneous Candidiasis: IL-17RA Deficiency

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  • First Online:
Encyclopedia of Medical Immunology

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Synonyms

Candidiasis familial 5 (CANDF5); IL-17R deficiency; Immunodeficiency 51 (IMD51)

Definition

Interleukin-17 receptor A (IL-17RA) deficiency is an autosomal recessive disorder that leads to chronic mucocutaneous candidiasis (CMC). CMC is defined as recurrent or persistent mucocutaneous infections caused by fungi of the genus Candida and primarily by the commensal organism Candida albicans (Kirkpatrick 2001; Puel et al. 2012). IL-17RA deficiency leads to CMC through impairments in IL-17-mediated immunity.

Introduction

Interleukin-17 (IL-17) is a pro-inflammatory cytokine that plays a critical role in antimicrobial defense. First identified in the 1990s, both IL-17 and the IL-17 receptor were striking in their unique protein structure, bearing no resemblance to other known cytokines or cytokine receptors (Aggarwal and Gurney 2002; Rouvier et al. 1993; Yao et al. 1995). Since then, the importance of this distinct family of cytokines in immunity has become well established. IL-17...

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Author information

Authors and Affiliations

  1. Department of Pediatrics, BC Children’s Hospital, University of British Columbia, Vancouver, BC, Canada

    Catherine M. Biggs & Stuart E. Turvey

Authors
  1. Catherine M. Biggs
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  2. Stuart E. Turvey
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Corresponding author

Correspondence to Catherine M. Biggs .

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. British Columbia Children’s Hospital, University of British Columbia, Vancouver, BC, Canada

    Stuart E Turvey

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Biggs, C.M., Turvey, S.E. (2018). Chronic Mucocutaneous Candidiasis: IL-17RA 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_60-1

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  • DOI: https://doi.org/10.1007/978-1-4614-9209-2_60-1

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  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4614-9209-2

  • Online ISBN: 978-1-4614-9209-2

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