Improvement of polymorphonuclear leucocyte oxidative and bactericidal functions in chronic granulomatous disease with 4-amino-4'-hydroxylaminodiphenyl sulphone

British Journal of Haematology
G IsmailR L Baehner


In an effort to restore oxidant-dependent capabilities to chronic granulomatous disease (CGD) polymorphonuclear leucocytes (PMN), we studied a dapsone derivative, 4-amino-4'-hydroxylaminodiphenyl sulphone (DDS-NOH), known to generate H2O2. After incubation of CGD PMN with 0.2 and 1.0 mM DDS-NOH for 30 min, the rate of glucose-1-14C oxidation via hexose monophosphate (HMP) shunt increased 2--4-fold and that of iodination of ingested zymosan particles 1.5--2.7-fold. Both effects could be further enhanced by superoxide dismutase (SOD) but inhibited by catalase. In three patients, 0.2 mM DDS-NOH improved in vitro killing of Staph. aureus. DDS-NOH 0.02 mM induced capping of Concanavalin A (Con A) receptor complexes suggesting interference by the drug with microtubule-associated function. Thus, optimal concentrations of DDS-NOH may be employed as an oxidant to improve metabolic and bactericidal functions of PMN from patients with CGD.


Jun 12, 1975·European Journal of Clinical Investigation·J HakimJ Boucherot
Aug 1, 1975·Archives of Biochemistry and Biophysics·K Takanaka, P J O'Brien
Feb 1, 1977·The Journal of Clinical Investigation·I M GoldsteinH B Kaplan
Apr 1, 1975·The Journal of Clinical Investigation·D C Hohn, R I Lehrer
Oct 1, 1975·The Journal of Clinical Investigation·B M BabiorB S Kipnes
Aug 1, 1975·Archives of Biochemistry and Biophysics·K Takanaka, P J O'Brien
Mar 14, 1974·The New England Journal of Medicine·J T CurnutteB M Babior
May 2, 1968·The New England Journal of Medicine·R L Baehner, D G Nathan
Apr 1, 1970·The Journal of Clinical Investigation·R L BaehnerM L Karnovsky
Feb 27, 1969·The New England Journal of Medicine·S J Klebanoff, L R White
May 1, 1970·The Journal of Clinical Investigation·R L BaehnerM L Karnovsky
Aug 1, 1946·Journal of the American Chemical Society·E L JACKSON


Jan 1, 1981·Journal of Immunological Methods·A G White, S M Walker
Mar 1, 1983·Veterinary Immunology and Immunopathology·G S Smith, J H Lumsden
Oct 1, 1984·Antimicrobial Agents and Chemotherapy·W T Hughes, B L Smith
Jul 1, 1980·British Journal of Haematology·R M WeetmanR L Baehner
Jul 1, 1986·Human Toxicology·D B HutchinsonJ A Farquhar
Dec 7, 2016·Surgical Neurology International·Hiroshi TakasunaYuichiro Tanaka

Related Concepts

Aminobiphenyl Compounds
Blood Bactericidal Activity
Catalase T
Concanavalin A
Anhydrous Dextrose
Autosomal Recessive Chronic Granulomatous Disease
Immunologic Capping
Neutrophil Band Cells
Ag-Zn Superoxide Dismutase

Trending Feeds


Coronaviruses encompass a large family of viruses that cause the common cold as well as more serious diseases, such as the ongoing outbreak of coronavirus disease 2019 (COVID-19; formally known as 2019-nCoV). Coronaviruses can spread from animals to humans; symptoms include fever, cough, shortness of breath, and breathing difficulties; in more severe cases, infection can lead to death. This feed covers recent research on COVID-19.

Evolution of Pluripotency

Pluripotency refers to the ability of a cell to develop into three primary germ cell layers of the embryo. This feed focuses on the mechanisms that underlie the evolution of pluripotency. Here is the latest research.

Lipidomics & Rhinovirus Infection

Lipidomics can be used to examine the lipid species involved with pathogenic conditions, such as viral associated inflammation. Discovered the latest research on Lipidomics & Rhinovirus Infection.

Alzheimer's Disease: MS4A

Variants within the membrane-spanning 4-domains subfamily A (MS4A) gene cluster have recently been implicated in Alzheimer's disease in genome-wide association studies. Here is the latest research on Alzheimer's disease and MS4A.

Chronic Fatigue Syndrome

Chronic fatigue syndrome is a disease characterized by unexplained disabling fatigue; the pathology of which is incompletely understood. Discover the latest research on chronic fatigue syndrome here.

Torsion Dystonia

Torsion dystonia is a movement disorder characterized by loss of control of voluntary movements appearing as sustained muscle contractions and/or abnormal postures. Here is the latest research.

Generating Insulin-Secreting Cells

Reprogramming cells or using induced pluripotent stem cells to generate insulin-secreting cells has significant therapeutic implications for diabetics. Here is the latest research on generation of insulin-secreting cells.

Central Pontine Myelinolysis

Central Pontine Myelinolysis is a neurologic disorder caused most frequently by rapid correction of hyponatremia and is characterized by demyelination that affects the central portion of the base of the pons. Here is the latest research on this disease.

Epigenome Editing

Epigenome editing is the directed modification of epigenetic marks on chromatin at specified loci. This tool has many applications in research as well as in the clinic. Find the latest research on epigenome editing here.