People With This Rare Disease Can Barely Eat Protein.  Biotechnology May Change That.

People With This Rare Disease Can Barely Eat Protein.  Biotechnology May Change That.

The Brown family at the Grand Tetons (2019). Clockwise from left, Christine, Kevin, Keagan, Connor, and Kellen.

Courtesy Brown family

Imagine that the protein in bread, eggs, steak, even beans is not the foundation for a healthy diet, but a poison to your brain. That is the reality for people living with Phenylketonuria, or PKU. This cluster of rare genetic variations affects the ability to digest phenylalanine (Phe), one of the chemical building blocks of protein. The toxins can build up in the brain causing severe mental retardation.

Can a probiotic help digest the troublesome proteins before they can enter the bloodstream and travel to the brain? A Boston area biotech start up, Synlogic, believes it can. Their starting point is an E. coli bacterium that has been used as a probiotic for more than a century. The company then screened thousands of gene variants to identify ones that produced enzymes most efficient at slicing and dicing the target proteins and optimized them further through directed evolution. The results have been encouraging.

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Bob Roehr
Bob Roehr is a biomedical journalist based in Washington, DC. Over the last twenty-five years he has written extensively for The BMJ, Scientific American, PNAS, Proto, and myriad other publications. He is primarily interested in HIV, infectious disease, immunology, and how growing knowledge of the microbiome is changing our understanding of health and disease. He is working on a book about the ways the body can at least partially control HIV and how that has influenced (or not) the search for a treatment and cure.
After his grandmother’s dementia diagnosis, one man invented a snack to keep her healthy and hydrated.

Founder Lewis Hornby and his grandmother Pat, sampling Jelly Drops—an edible gummy containing water and life-saving electrolytes.

jellydrops.us

On a visit to his grandmother’s nursing home in 2016, college student Lewis Hornby made a shocking discovery: Dehydration is a common (and dangerous) problem among seniors—especially those that are diagnosed with dementia.

Hornby’s grandmother, Pat, had always had difficulty keeping up her water intake as she got older, a common issue with seniors. As we age, our body composition changes, and we naturally hold less water than younger adults or children, so it’s easier to become dehydrated quickly if those fluids aren’t replenished. What’s more, our thirst signals diminish naturally as we age as well—meaning our body is not as good as it once was in letting us know that we need to rehydrate. This often creates a perfect storm that commonly leads to dehydration. In Pat’s case, her dehydration was so severe she nearly died.

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Sarah Watts

Sarah Watts is a health and science writer based in Chicago.

A vaccine for ovarian cancer is now in development

Theupcoming vaccine is changing the way we look at treating one of the country’s deadliest cancers.

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Last week, researchers at the University of Oxford announced that they have received funding to create a brand new way of preventing ovarian cancer: A vaccine. The vaccine, known as OvarianVax, will teach the immune system to recognize and destroy mutated cells—one of the earliest indicators of ovarian cancer.


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Sarah Watts

Sarah Watts is a health and science writer based in Chicago.