Infectious diseases in patients with IRAK-4, MyD88, NEMO, or IκBα deficiency

C Picard, JL Casanova, A Puel - Clinical microbiology reviews, 2011 - Am Soc Microbiol
Autosomal recessive IRAK-4 and MyD88 deficiencies predispose affected patients to
recurrent invasive pyogenic bacterial infection. Both defects result in the selective …

Role of the innate immune system in host defence against bacterial infections: focus on the Toll‐like receptors

B Albiger, S Dahlberg… - Journal of internal …, 2007 - Wiley Online Library
The innate immunity plays a critical role in host protection against pathogens and it relies
amongst others on pattern recognition receptors such as the Toll‐like receptors (TLRs) and …

Selective predisposition to bacterial infections in IRAK-4–deficient children: IRAK-4–dependent TLRs are otherwise redundant in protective immunity

CL Ku, H Von Bernuth, C Picard, SY Zhang… - The Journal of …, 2007 - rupress.org
Human interleukin (IL) 1 receptor–associated kinase 4 (IRAK-4) deficiency is a recently
discovered primary immunodeficiency that impairs Toll/IL-1R immunity, except for the Toll …

Human TLR-7-,-8-, and-9-mediated induction of IFN-α/β and-λ is IRAK-4 dependent and redundant for protective immunity to viruses

K Yang, A Puel, S Zhang, C Eidenschenk, CL Ku… - Immunity, 2005 - cell.com
Five TLRs are thought to play an important role in antiviral immunity, sensing viral products
and inducing IFN-α/β and-λ. Surprisingly, patients with a defect of IRAK-4, a critical kinase …

Human genetics of infectious diseases: a unified theory

JL Casanova, L Abel - The EMBO journal, 2007 - embopress.org
Since the early 1950s, the dominant paradigm in the human genetics of infectious diseases
postulates that rare monogenic immunodeficiencies confer vulnerability to multiple infectious …

Complement deficiency and disease: an update

AG Sjöholm, G Jönsson, JH Braconier, G Sturfelt… - Molecular …, 2006 - Elsevier
Complement deficiencies are probably vastly under-diagnosed within clinical medicine.
Judging from a Swedish study of C2 deficiency, a deficiency with an estimated prevalence of …

Genetic deficiencies of innate immune signalling in human infectious disease

E van de Vosse, JT van Dissel… - The Lancet infectious …, 2009 - thelancet.com
Summary The type-1 cytokine (interleukin 12, interleukin 23, interferon γ, interleukin 17)
signalling pathway is triggered during infection by activation of phagocyte-expressed pattern …

IRAK4 and NEMO mutations in otherwise healthy children with recurrent invasive pneumococcal disease

CL Ku, C Picard, M Erdös, A Jeurissen… - Journal of medical …, 2007 - jmg.bmj.com
Background: About 2% of childhood episodes of invasive pneumococcal disease (IPD) are
recurrent, and most remain unexplained. Objective: To report two cases of otherwise …

Translational science and the hidden research system in universities and academic hospitals: A case study

B Lander, J Atkinson-Grosjean - Social Science & Medicine, 2011 - Elsevier
Innovation systems (IS) and science policy scholarship predominantly focus on linkages
between universities and industry, and the commercial translation of academic discoveries …

Identification of high and low responders to lipopolysaccharide in normal subjects: an unbiased approach to identify modulators of innate immunity

MM Wurfel, WY Park, F Radella, J Ruzinski… - The Journal of …, 2005 - journals.aai.org
LPS stimulates a vigorous inflammatory response from circulating leukocytes that varies
greatly from individual to individual. The goal of this study was to use an unbiased approach …