Genetic defect may confer resistance to certain viral infections

April 9, 2014, NIH/National Institute of Allergy and Infectious Diseases
Viruses (blue and pink) must use host cells (green) to create more viruses that spread infection (top panel). CDG-IIb patients have defective glycosylation, the process of adding sugars to proteins, resulting in poor production of viruses that depend on this process, such as HIV and influenza (bottom panel). The scientists also show that viruses coming from the patients' cells (orange) are less infectious because of changes to their outer shields. Credit: NIAID

A National Institutes of Health (NIH) study reports that a rare genetic disease, while depleting patients of infection-fighting antibodies, may actually protect them from certain severe or recurrent viral infections. Researchers found that HIV and influenza viruses replicate in the cells of people with congenital disorder of glycosylation type IIb (CDG-IIb) at a much lower rate than in healthy donor cells, creating fewer and less infectious viruses. The study, published in The New England Journal of Medicine, was led by Sergio Rosenzweig, M.D., Ph.D., director of the Primary Immune Deficiency (PID) Clinic at the NIH's National Institute of Allergy and Infectious Diseases (NIAID).

In the study, the researchers diagnosed CDG-IIb in two siblings with severe development issues who were referred to the NIAID PID Clinic though the NIH Undiagnosed Diseases Program. CDG-IIb is extremely rare, with only one other case reported. The genetic defect of the disease disrupts glycosylation, or the process of attaching sugars to proteins. As a result, proteins called gamma globulins, which include infection-fighting antibodies, are unstable and persist at low levels in the patients' blood.

Interestingly, some viruses, including HIV and influenza, depend on glycosylation to form protective shields. The researchers showed that these were less able to replicate or create protective shields because of the glycosylation defects in CDG-IIb cells. In comparison, adenovirus, poliovirus and vaccinia virus, which either do not rely on glycosylation or do not form protective shields, replicated normally when added to both CDG-IIb and healthy cells. This study suggests that modulating aspects of host glycosylation may be a strategy to control certain .

Explore further: Research team identifies new genetic syndrome

More information: MA Sadat, S Moir et al. Glycosylation, hypogammaglobulinemia and resistance to viral infections. NEJM, DOI: 10.1056/NEJMoa1302846 (2014).

Related Stories

Research team identifies new genetic syndrome

March 4, 2014
Researchers at the National Institutes of Health (NIH) have identified a new genetic syndrome characterized by a constellation of health problems, including severe allergy, immune deficiency, autoimmunity and motor and neurocognitive ...

A cautionary tale on genome-sequencing diagnostics for rare diseases

May 10, 2013
Children born with rare, inherited conditions known as Congenital Disorders of Glycosylation, or CDG, have mutations in one of the many enzymes the body uses to decorate its proteins and cells with sugars. Properly diagnosing ...

Holy glycosylation! New 'bat signal' flags distressed cells in childhood genetic diseases

June 12, 2012
Just as Gotham City uses the Bat Signal to call for Batman's aid, a new tool developed by scientists from the Sanford-Burnham Medical Research Institute in La Jolla, California, should serve as the cellular equivalent for ...

Trick that aids viral infection is identified

January 30, 2014
Scientists have identified a way some viruses protect themselves from the immune system's efforts to stop infections, a finding that may make new approaches to treating viral infections possible.

Severe congenital disorder successfully treated in a mouse model for the first time

December 22, 2011
Using a mouse model, Heidelberg University Hospital researchers have for the first time successfully treated a severe congenital disorder in which sugar metabolism is disturbed. The team headed by Prof. Christian Korner, ...

NIH study describes new method for tracking T cells in HIV patients

February 3, 2014
A team of researchers has reported a novel method for tracking CD4+ T cells in people infected with HIV. CD4+ T cells are critical for immune defense against an array of pathogens and are a primary target of HIV. In the study, ...

Recommended for you

Forces from fluid in the developing lung play an essential role in organ development

January 23, 2018
It is a marvel of nature: during gestation, multiple tissue types cooperate in building the elegantly functional structures of organs, from the brain's folds to the heart's multiple chambers. A recent study by Princeton researchers ...

More surprises about blood development—and a possible lead for making lymphocytes

January 22, 2018
Hematopoietic stem cells (HSCs) have long been regarded as the granddaddy of all blood cells. After we are born, these multipotent cells give rise to all our cell lineages: lymphoid, myeloid and erythroid cells. Hematologists ...

How metal scaffolds enhance the bone healing process

January 22, 2018
A new study shows how mechanically optimized constructs known as titanium-mesh scaffolds can optimize bone regeneration. The induction of bone regeneration is of importance when treating large bone defects. As demonstrated ...

Researchers illustrate how muscle growth inhibitor is activated, could aid in treating ALS

January 19, 2018
Researchers at the University of Cincinnati (UC) College of Medicine are part of an international team that has identified how the inactive or latent form of GDF8, a signaling protein also known as myostatin responsible for ...

Bioengineered soft microfibers improve T-cell production

January 18, 2018
T cells play a key role in the body's immune response against pathogens. As a new class of therapeutic approaches, T cells are being harnessed to fight cancer, promising more precise, longer-lasting mitigation than traditional, ...

Weight flux alters molecular profile, study finds

January 17, 2018
The human body undergoes dramatic changes during even short periods of weight gain and loss, according to a study led by researchers at the Stanford University School of Medicine.

0 comments

Please sign in to add a comment. Registration is free, and takes less than a minute. Read more

Click here to reset your password.
Sign in to get notified via email when new comments are made.