Study linking idiopathic pulmonary fibrosis, a deadly human disease with no effective treatment options, to presence of a monkey virus

Date: Nov-19-2013Enzo Biochem Inc. (NYSE:ENZ) has announced publication of a study in a leading scientific journal, Modern Pathology, a Nature Publishing Group publication, that shows a strong association of idiopathic pulmonary fibrosis (IPF), a pulmonary disease with 100% mortality within five years, with the presence of the herpesvirus saimiri virus, a virus native to squirrel monkeys. The breakthrough discovery of the IPF's origin is expected to result in a clinical diagnostic that could lead to screening and diagnosis, and perhaps result in potential treatment, for this fatal disease.

The publication was authored by scientists from Enzo Biochem, Ohio State University, the Medical College of Wisconsin and Cornell University. The discovery is the subject of a patent application that is exclusively assigned to Enzo Biochem. "The significance of this discovery, beyond providing a reliable marker for screening and diagnosis, is the potential for development of new therapeutic strategies and better understanding of the progression of the disease in humans," said Elazar Rabbani, study co-author and Chief Executive Officer of Enzo. "This may also translate into a patient's ability to live with the disease or even be cured if the fibrosis is not too advanced."

Pulmonary fibrosis affects about one million people in the United States, of which IPF represents about 200,000 cases (20%). There is currently no effective way to diagnose IPF prior to the clinical manifestation of symptoms and treatments are largely ineffective. This new marker for IPF could address the urgent critical requirement for early diagnosis and screening of the pulmonary fibrosis population (over a million subjects).

"Discovering the origin of IPF gives us hope that both diagnostics and treatments will be developed to help patients survive and live with this devastating illness," said Gerard J. Nuovo, MD, study co-author and Professor (retired), Ohio State University Comprehensive Cancer Center. "While the sample size is small there are multiple data points that support our findings that herpesvirus saimiri infection may be the cause of IPF. For example, there was a 100% correlation  - both positively and negatively-where the virus was present in all IPF samples and absent in all non-IPF controls. Additionally, the virus was found in the cell that orchestrates IPF and a key gene expressed in the tissue was identified as viral, and not human, in nature."

Methodology and Findings

The goal of the study was to evaluate the role of the herpesviruses family in IPF. Researchers used 21 paraffin embedded lung biopsies from patients diagnosed with IPF, and 21 lung biopsies from age matched controls with pulmonary fibrosis that is not IPF.

DNA studies demonstrated that two labeled probes derived from the herpesvirus saimiri genome reacted with the DNA in regenerating epithelial cells in 21/21 IPF biopsies and didn't react with DNA in 21/21 of the control samples. Subsequent immunological evaluation found that four proteins known to be expressed from the genome of various herpesviruses (cyclin D, thymidylate synthase, dihydrofolate reductase, and interleukin-17) were strongly co-expressed in the regenerating epithelial cells of each of the 21 idiopathic pulmonary fibrosis cases, and not in the benign epithelia of the controls. Among herpesviruses, only herpesvirus saimiri is known to express all four proteins. Future research efforts will also be directed towards investigations of other diseases, for any possible role of this virus in their etiology.

Idiopathic pulmonary fibrosis (IPF) is a chronic and ultimately fatal disease characterized by a progressive decline in the ability of the lungs to function. The disease progresses by thickening the lining of the lungs via scarring, causing an irreversible loss of the tissue's ability to transport oxygen. Symptoms include dry, persistent cough lasting longer than a month and chronic shortness of breath. IPF ultimately robs the sufferer of the ability to breathe. Approximately 200,000 people, or 20% of all pulmonary fibrosis patients, have an idiopathic form of the disease. Mortality is almost 100% within five years after diagnosis.

About Herpesviruses

Herpesviruses are a large family of DNA viruses known to cause diseases in animals. All herpesviruses are nuclear-replicating - the viral DNA is transcribed to mRNA within the nucleus of the infected cell. Herpesvirus saimiri is present in virtually all squirrel monkeys, and in its natural host, the virus does not cause disease. However, infection of other monkey species such as tamarins, marmosets and owl monkeys leads to a fatal T-cell lymphoma. In addition, a related mouse Herpesvirus, MuHV4, is used as an animal model for IPF.

Courtesy: Medical News Today Note: Any medical information available in this news section is not intended as a substitute for informed medical advice and you should not take any action before consulting with a health care professional.