New Study Suggests Activity of Propranolol in Hypotrichosis-Lymphedema-Telangiectasia Patients

According to a publication from ScienceDaily, a new study of propranolol suggests explanations for its ability to shrink certain benign tumors in infants and relieve symptoms of hypotrichosis-lymphedema-telangiectasia syndrome.

About Hypotrichosis-Lymphedema-Telangiectasia Syndrome

Hypotrichosis-lymphedema-telangiectasia syndrome (HLTS) is a highly rare condition characterized by (as its name would imply) sparseness of hair (hypotrichosis), lymph buildup within the body (lymphedema), and the development of narrow blood vessel patterns on the surface of the skin (telangiectasia). Almost all HLTS patients lack eyebrows and lashes, and develop alopecia and lymphatic abnormalities.

The condition is thought to be caused by mutations to a gene called SOX18 (No word yet if the researcher to name the gene was a clairvoyant Red Sox fan). In healthy individuals, SOX18 codes for the production of the SOX18 protein, which is involved in protein transcription. The larger SOX protein family is involved in embryonic development and cell differentiation. SOX18 is known to be involved in the normal development of hair, blood vessels, and lymphatic vessels.

Currently, there is no cure for HLTS. Treatment options are varied, however, and can be tailored to meet a specific patient’s needs.

New Findings About Propranolol

Propranolol is a commonly prescribed beta-blocker primarily administered to lower blood pressure. It has a huge variety of uses, however, and is also used to treat irregular heartbeat, tremors, anxiety, chest pain, and more.

Propranolol works by binding to adrenaline receptors, limiting the uptake of adrenaline. In more recent years, it’s also been used to shrink benign skin tumors in infants called hemangiomas, Researchers weren’t sure why the tumors were shrinking, though.

A study at Boston Children’s Hospital now offers an explanation. Earlier, an adolescent HLTS patient with “unusually mild” symptoms had been described in medical literature. The patient had taken propranolol from an early age to limit high blood pressure.

In another study, researchers found that propranolol controlled SOX18 activity by stopping it from linking with other SOX18 proteins. An animal study of mice found that the drug limited symptoms similar to those expressed in HLTS.

The Boston Children’s Hospital research team finally had the basis for a study. They sampled hemangioma cells from living patients, and replicated them in a lab. When propranolol was introduced to the hemangiomas, it stopped nascent cells from evolving into tumor cells. One part of the drug, the R(+) enantiomer, was specifically found to inhibit SOX18 activity.

The findings suggest that it may be possible to treat “hemangiomas or HLTS using only the R(+) enantiomer” of the drug, said the study’s co-senior author Dr. Mathias Francois. The finding may also lead to improved understanding of vascular diseases in the future.

The potential uses of propranolol are highly varied and only growing more so. What do you think of this exciting finding? Share your thoughts with Patient Worthy!

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