Navigation Links
Molecular interplay explains many immunodeficiencies
Date:11/11/2013

Australian scientists have described an exquisitely balanced interplay of four molecules that trigger and govern antibody production in immune cells. As well as being an important basic science discovery, it helps explain why people with mutations in any one of the associated genes cannot fight infection effectively, and develop rare and crippling immunodeficiency disorders.

Our immune system is made of a number of different types of cells that undertake specific functions. Those that make antibodies are known as 'B cells', and they become active after infection. Once a B cell is activated, it can proliferate into thousands of clones, known as 'plasma cells', which patrol the body and secrete large amounts of antibody to destroy the invader.

Dr Lucinda Berglund and Associate Professor Stuart Tangye, from Sydney's Garvan Institute of Medical Research, are the first to describe a specific molecular process that controls the activation and differentiation of B cells. They used human blood and tissue samples to show that the chemical messaging molecule interleukin 21 (IL-21) activates the STAT3 gene in B cells, which in turn triggers the expression of a molecule known as 'CD25', a cell surface receptor that attracts a second messaging molecule, interleukin 2 (IL-2). IL-21 and IL-2 then work co-operatively to induce plasma cell development and antibody production. Their findings are published in the international journal Blood, now online.

"The interesting and informative aspect of this finding for me is that some people have mutations in the IL-21 receptor, some have mutations in STAT3, while others have mutations in CD25, and they all have B cell defects," said Associate Professor Tangye.

"By examining B cells from people with specific genetic mutations, we revealed that both components of the IL-21 receptor are critical for B cell function and people can have mutations in either, with equally debilitating effects. We see these effects in patients with X-linked severe combined immunodeficiency, whose impaired response to IL-21 causes severe antibody deficiency."

"Patients with mutations in the STAT3 gene develop Hyper IgE Syndrome, another rare immunodeficiency that manifests as compromised antibody production and greatly depleted immune defences."

Immunodeficiencies arising from mutations in single genes give scientists a unique opportunity to understand B cell signaling, and reveal potential targets for modulating B cell responses in immunodeficiency and autoimmunity.

The current study arose from analysing global gene expression in B cells from healthy people and people with STAT3 deficiency which immediately highlighted genes that were poorly expressed in disease. The Tangye lab plans to investigate other genes that impact the function of B cells.


'/>"/>

Contact: Alison Heather
a.heather@garvan.org.au
61-292-958-128
Garvan Institute of Medical Research
Source:Eurekalert

Related biology news :

1. The Association for Molecular Pathology announces highlights of Phoenix meeting
2. Penn researchers identify molecular link between gut microbes and intestinal health
3. Singapore scientists expose molecular secrets of bile duct cancers from different countries
4. Molecular Velcro may lead to cost-effective alternatives to natural antibodies
5. Grafted limb cells acquire molecular fingerprint of new location, UCI study shows
6. Molecular biology: Designer of protein factories exposed
7. The molecular clock of the common buzzard
8. Cells molecular muscles help them sense and respond to their environments
9. Trovagene Announces Abstracts to be Presented at Molecular Targets and Cancer Therapeutics Conference
10. Recent highlights in Molecular Biology and Evolution
11. Better insight into molecular interactions
Post Your Comments:
*Name:
*Comment:
*Email:
(Date:2/2/2016)... NEW YORK , Feb. 2, 2016 /PRNewswire/ ... Potentials of that Rising Market Are you ... new analysis forecasts revenues for checkpoint inhibitors. Visiongain,s ... world market, submarket, product and national level. ... Instead discover what progress, opportunities and revenues those ...
(Date:2/1/2016)... BURNABY, Canada , February 1, 2016 ... new technological advancements to drive global touchfree intuitive gesture ...   --> Rising sales of consumer electronics ... intuitive gesture control market size ... of consumer electronics coupled with new technological advancements to ...
(Date:1/27/2016)... 2016  Rite Track, Inc. a leading semiconductor equipment ... Chester, Ohio announced today the acquisition of PLUS ... in Austin, Texas , will significantly ... modifications, installations and technical support offerings for TEL Track ... commented, "PLUS has provided world class service including refurbishment, ...
Breaking Biology News(10 mins):
(Date:2/9/2016)... , Feb. 9, 2016 This market ... the current and future prospects of the market in ... report include companies engaged in the manufacture of microbiology ... executive summary with a market snapshot providing the overall ... of this report. This section also provides the overall ...
(Date:2/9/2016)... , February 9, 2016 Three-Year ... Enables Children to Take Part in Life-Changing Camp ... new initiative designed to positively affect the lives of children born ... care. --> SHPG ) is announcing a new initiative ... diseases, as well as the future of rare disease care. ...
(Date:2/9/2016)... LONDON , Feb. 9, 2016 /PRNewswire/ ... (PTP1B) Inhibitors-Pipeline Insights, 2016", report provides in ... their development activities around the Protein-Tyrosine Phosphatase ... the product profiles in various stages of ... Phase II, Phase III and Preregistration. Report ...
(Date:2/8/2016)... 2016 Should antibiotic bone cement products be ... prevent infection after standard total hip or knee replacement ... Institute have been fielding a lot lately. ... Line?" --> "Antibiotic Bone Cement: ... --> While there isn,t a simple answer, ...
Breaking Biology Technology: