Literature DB >> 31172494

Chronic Granulomatous Disease.

Dirk Roos1.   

Abstract

Chronic granulomatous disease is a clinical condition that stems from inactivating mutations in NOX2 and its auxiliary proteins. Together, these proteins form the phagocyte NADPH oxidase enzyme that generates superoxide. Superoxide (O2ċ-) and its reduced product hydrogen peroxide (H2O2) give rise to several additional reactive oxygen species (ROS), which together are necessary for adequate killing of pathogens. Thus, CGD patients, with a phagocyte NADPH oxidase that is not properly functioning, suffer from recurrent, life-threatening infections with certain bacteria, fungi, and yeasts. Here, I give a short survey of the genetic mutations that underlie CGD, the effect of these mutations on the activity of the leukocyte NADPH oxidase, the clinical symptoms of CGD patients, and the treatment options for these patients.

Entities:  

Keywords:  CYBA; CYBB; Chronic granulomatous disease; Mutations; NADPH oxidase; NCF1; NCF2; NCF4; NOX2; Phagocytes

Mesh:

Substances:

Year:  2019        PMID: 31172494     DOI: 10.1007/978-1-4939-9424-3_32

Source DB:  PubMed          Journal:  Methods Mol Biol        ISSN: 1064-3745


  6 in total

1.  Skeletons in the Cupboard of Dysfunctional Neutrophils Revealed.

Authors:  Heiko Herwald; Arne Egesten
Journal:  J Innate Immun       Date:  2021-01-21       Impact factor: 7.349

2.  Skin manifestations in pediatric patients with primary immunodeficiency diseases (PIDs) in a tertiary care hospital in Colombia.

Authors:  William López-Quintero; Daniela Cleves; Jose David Gomez-Vasco; Paola Pérez; Jaime Patiño; Diego Medina-Valencia; Harry Pachajoa; Laura Torres-Canchala; Andres Vidal; Manuela Olaya
Journal:  World Allergy Organ J       Date:  2021-03-01       Impact factor: 4.084

Review 3.  Precise somatic genome editing for treatment of inborn errors of immunity.

Authors:  Qingzhou Meng; Haixiang Sun; Jianghuai Liu
Journal:  Front Immunol       Date:  2022-08-26       Impact factor: 8.786

4.  Deep RNA sequencing of intensive care unit patients with COVID-19.

Authors:  Alger M Fredericks; Maximilian S Jentzsch; William G Cioffi; Maya Cohen; William G Fairbrother; Shivam J Gandhi; Elizabeth O Harrington; Gerard J Nau; Jonathan S Reichner; Corey E Ventetuolo; Mitchell M Levy; Alfred Ayala; Sean F Monaghan
Journal:  Sci Rep       Date:  2022-09-21       Impact factor: 4.996

Review 5.  NADPH oxidases: Current aspects and tools.

Authors:  Katrin Schröder
Journal:  Redox Biol       Date:  2020-05-23       Impact factor: 11.799

6.  Functional Defect of Neutrophils Causing Dermatophytosis: Case Report.

Authors:  Rosemeire N Constantino-Silva; Sandro F Perazzio; Nicolas de Albuquerque Weidebach; Anete S Grumach
Journal:  J Fungi (Basel)       Date:  2020-10-22
  6 in total

北京卡尤迪生物科技股份有限公司 © 2022-2023.