Mendelian susceptibility to mycobacterial disease: genetic, immunological, and clinical features of inborn errors of IFN-γ immunity

J Bustamante, S Boisson-Dupuis, L Abel… - Seminars in …, 2014 - Elsevier
Mendelian susceptibility to mycobacterial disease (MSMD) is a rare condition characterized
by predisposition to clinical disease caused by weakly virulent mycobacteria, such as BCG …

Gene therapy on the move

KB Kaufmann, H Büning, A Galy… - EMBO molecular …, 2013 - embopress.org
The first gene therapy clinical trials were initiated more than two decades ago. In the early
days, gene therapy shared the fate of many experimental medicine approaches and was …

[HTML][HTML] Cancers related to immunodeficiencies: update and perspectives

E Mortaz, P Tabarsi, D Mansouri, A Khosravi… - Frontiers in …, 2016 - frontiersin.org
The life span of patients with primary and secondary immunodeficiency is increasing due to
recent improvements in therapeutic strategies. While the incidence of primary …

Human TLRs and IL-1Rs in host defense: natural insights from evolutionary, epidemiological, and clinical genetics

JL Casanova, L Abel… - Annual review of …, 2011 - annualreviews.org
Toll-like receptors (TLRs) and interleukin-1 receptors (IL-1Rs) have TIR intracellular
domains that engage two main signaling pathways, via the TIR-containing adaptors MyD88 …

Are complement deficiencies really rare? Overview on prevalence, clinical importance and modern diagnostic approach

AS Grumach, M Kirschfink - Molecular immunology, 2014 - Elsevier
Complement deficiencies comprise between 1 and 10% of all primary immunodeficiencies
(PIDs) according to national and supranational registries. They are still considered rare and …

Laboratory evaluation of primary immunodeficiencies

JB Oliveira, TA Fleisher - Journal of Allergy and Clinical Immunology, 2010 - Elsevier
Primary immunodeficiencies are congenital disorders caused by defects in different
elements of the immune system. Affected patients usually present clinically with recurrent …

Primary B-cell immunodeficiencies

T Smith, C Cunningham-Rundles - Human immunology, 2019 - Elsevier
Primary B-cell immunodeficiencies refer to diseases resulting from impaired antibody
production due to either molecular defects intrinsic to B-cells or a failure of interaction …

The mucosal firewalls against commensal intestinal microbes

AJ Macpherson, E Slack, MB Geuking… - Seminars in …, 2009 - Springer
Mammals coexist with an extremely dense microbiota in the lower intestine. Despite the
constant challenge of small numbers of microbes penetrating the intestinal surface …

[图书][B] Textbook of Interdisciplinary Pediatric Palliative Care: Expert Consult Premium Edition-Enhanced Online Features and Print

J Wolfe, P Hinds, B Sourkes - 2011 - books.google.com
The Textbook of Interdisciplinary Pediatric Palliative Care, by Drs. Joanne Wolfe, Pamela
Hinds, and Barbara Sourkes, aims to inform interdisciplinary teams about palliative care of …

Enabling a systems biology approach to immunology: focus on innate immunity

JL Gardy, DJ Lynn, FSL Brinkman, REW Hancock - Trends in immunology, 2009 - cell.com
Immunity is not simply the product of a series of discrete linear signalling pathways; rather it
is comprised of a complex set of integrated responses arising from a dynamic network of …