Understanding B Cells - To React Or Not To React

December 03, 1997

X-linked agammaglobulinemia (XLA) is a hereditary disease which affects one in every 150,000 boys born worldwide each year. Boys with the condition have no B cells, the white blood cells which produce antibodies, in their blood. Unsurprisingly they are very susceptible to infection and require continuous medical treatment. At the British Society for Immunology Annual Congress in Brighton this week Dr Rudi Hendriks of the Erasmus University, Rotterdam, The Netherlands, will explain how he is trying to understand the condition using genetically modified mice.

The defective gene in XLA was identified in 1993 and has allowed the development of reliable methods for the genetic counselling of XLA families. The gene is necessary for the production of a cell signalling molecule called Bruton's tyrosine kinase (Btk). However, the role of Btk in B cell development was unclear.

To investigate its role, Dr Hendriks and his colleagues first produced some mice in which the Btk gene was inactive. They have been able to prove that the absence of the gene affects B cell development at the same stage in both mice and men.

Further investigation of the function of Btk has shown that it is essential when choosing which B cells launch an attack against invading bacteria. A B cell recognises foreign bacteria through receptors on its surface. However, once a bacterial protein has become attached to the receptor a signal has to be sent to the B cell's nucleus to tell it to react. This is where Btk comes in.

Intriguingly, Btk also seems to be important in telling a B cell not to react. B cells constantly encounter so-called "autoantigens". These are proteins produced by our own bodies. When the cells of the immune system encounter autoantigens there must be some mechanism which tells them that these antigens belong to the body itself and must not be attacked. Dr Hendriks believes that in the case of B cells, Btk plays a crucial role in this process. When the "do not attack" mechanism goes wrong, autoimmune diseases such as rheumatoid arthritis and diabetes can result. Dr Hendriks hopes that his research will, in the future, also contribute to a greater understanding of these diseases, by providing knowledge on the basic mechanism of self versus non-self recognition.

In the first instance, however, Dr Hendriks aims to help boys with XLA. In their mice, Dr Hendriks and his co-workers have been able to restore the B cells to full working order by inserting the human Btk gene. This suggests that gene therapy could one day allow these children, and their families, to lead normal lives.

Notes:


1. Dr Hendriks will be speaking in the Immune Deficiency session on Wednesday 3
December 1997. The BSI 5th Annual Congress will be held at the Brighton Centre,
Brighton, UK from 2-5 December.


2. Dr Hendriks can be contacted at the Department of Cell Biology and Genetics,
Erasmus University, Rotterdam, The Netherlands. Tel: + 31 10 4088 296 Fax: + 31 10 4360 225
hendriks@ch1.fgg.eur.nl


3. There will be a press office at the meeting in operation from 9am on Tuesday
2 December. Tel. +44 1 273 724 320 / 0378 406 416 Journalists are welcome to
attend but are asked to contact Kirstie Urquhart in advance to register.


4. Before the meeting Kirstie can be contacted on +44 181 875 2402 /
kirstie@immunology.org

British Society For Immunology

Related Rheumatoid Arthritis Articles from Brightsurf:

Reducing dementia in patients with rheumatoid arthritis
The incidence of dementia in patients with rheumatoid arthritis is lower in patients receiving biologic or targeted synthetic disease modifying antirheumatic drugs (DMARDs) than in patients who receive conventional synthetic DMARDs, according to a new study.

Is rheumatoid arthritis two different diseases?
While disease activity improves over time for most rheumatoid arthritis (RA) patients, long-term outcomes only improve in RA patients with autoantibodies, according to a new study published this week in PLOS Medicine by Xanthe Matthijssen of Leiden University Medical Center, Netherlands, and colleagues.

Does the Mediterranean diet protect against rheumatoid arthritis?
Previous research has demonstrated a variety of health benefits associated with the Mediterranean diet, which is rich in olive oil, cereals, fruit and vegetables, fish, and a moderate amount of dairy, meat, and wine.

Reducing corticosteroid use in rheumatoid arthritis
Is the long-term use of glucocorticoids essential in people with chronic inflammatory diseases such as rheumatoid arthritis, or can early discontinuation prevent characteristic side effects?

Rheumatoid arthritis patients under treatment with methotrexate
Patients with rheumatoid arthritis (RA) often suffer from what is referred to as interstitial lung disease (ILD).

Rheumatoid arthritis -- can its onset be delayed or prevented?
Rheumatoid arthritis (RA) is a chronic inflammatory autoimmune disorder that leads to significant health issues as well as high treatment costs.

Disease burden in osteoarthritis is similar to rheumatoid arthritis
Osteoarthritis (OA) has traditionally been viewed as a highly prevalent but milder condition when compared with rheumatoid arthritis (RA), and some may believe that it is part of a normal aging process requiring acceptance, not treatment.

Prospect of a new treatment for rheumatoid arthritis
An international research group led by Charité -- Universitätsmedizin Berlin has completed testing a new drug to treat rheumatoid arthritis.

Can rare lymphocytes combat rheumatoid arthritis?
Immunologists at Friedrich-Alexander-Universität Erlangen-Nürnberg have demonstrated that ILC2, a group of rare lymphoid cells, play a key role in the development of inflammatory arthritis.

Rheumatoid arthritis meets precision medicine
Scientists are bringing precision medicine to rheumatoid arthritis for the first time by using genetic profiling of joint tissue to see which drugs will work for which patients, reports a new multi-site study.

Read More: Rheumatoid Arthritis News and Rheumatoid Arthritis Current Events
Brightsurf.com is a participant in the Amazon Services LLC Associates Program, an affiliate advertising program designed to provide a means for sites to earn advertising fees by advertising and linking to Amazon.com.