Primary immunodeficiency disorders

Primary immunodeficiency disorders are a set of syndromes that arise from defects in the immune system. Most are diagnosed in young children, and patients are generally characterized by increased susceptibility to infections.

Latest Research and Reviews

  • Reviews |

    Advances in the design of vectors based on retroviruses, such as lentiviruses and gammaretroviruses, have led to improvements in the safety and stability of gene therapies directed at haematopoietic stem and progenitor cells. In this Review, Cavazzana and colleagues discuss the results from recent clinical trials of retroviral vectors for the treatment of genetic disorders, including severe combined immunodeficiencies and β-haemoglobinopathies (β-thalassaemia and sickle cell disease). They highlight the progress made and the remaining challenges in applying gene therapies more broadly.

    • Marina Cavazzana
    • , Frederic D. Bushman
    • , Annarita Miccio
    • , Isabelle André-Schmutz
    •  & Emmanuelle Six
  • Research |

    Zinc is important for normal immunity but its mechanistic actions are poorly understood. Hambleton and colleagues identify defects in Zn2+ transport that underpin a novel human immunodeficiency characterized by loss of mature B cells.

    • Consuelo Anzilotti
    • , David J. Swan
    • , Bertrand Boisson
    • , Mukta Deobagkar-Lele
    • , Catarina Oliveira
    • , Pauline Chabosseau
    • , Karin R. Engelhardt
    • , Xijin Xu
    • , Rui Chen
    • , Luis Alvarez
    • , Rolando Berlinguer-Palmini
    • , Katherine R. Bull
    • , Eleanor Cawthorne
    • , Adam P. Cribbs
    • , Tanya L. Crockford
    • , Tarana Singh Dang
    • , Amy Fearn
    • , Emma J. Fenech
    • , Sarah J. de Jong
    • , B. Christoffer Lagerholm
    • , Cindy S. Ma
    • , David Sims
    • , Bert van den Berg
    • , Yaobo Xu
    • , Andrew J. Cant
    • , Gary Kleiner
    • , T. Ronan Leahy
    • , M. Teresa de la Morena
    • , Jennifer M. Puck
    • , Ralph S. Shapiro
    • , Mirjam van der Burg
    • , J. Ross Chapman
    • , John C. Christianson
    • , Benjamin Davies
    • , John A. McGrath
    • , Stefan Przyborski
    • , Mauro Santibanez Koref
    • , Stuart G. Tangye
    • , Andreas Werner
    • , Guy A. Rutter
    • , Sergi Padilla-Parra
    • , Jean-Laurent Casanova
    • , Richard J. Cornall
    • , Mary Ellen Conley
    •  & Sophie Hambleton
    Nature Immunology 20, 350-361

News and Comment