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12:00 AM
 | 
Jan 03, 2011
 |  BioCentury  |  Emerging Company Profile

Hyperion: Building a better mop

Hyperion hopes HPN-100 prevents ammonia poisoning better than Buphenyl

The only drug approved as a chronic treatment for urea cycle disorders requires high doses and has high sodium content, resulting in a risk of hypertension and reduced appetite. Hyperion Therapeutics Inc. believes its sodium-free HPN-100 can avoid these side effects with reduced dosing intensity while achieving similar efficacy.

Ammonia is produced in the gut with protein ingestion. As part of the urea cycle in healthy patients, ammonia is converted to urea in the liver and later excreted.

Patients with urea cycle disorder (UCD) lack one of the six enzymes involved in urea metabolism. This hereditary error causes nitrogen to accumulate in the body in the form of ammonia, which can enter the brain and result in irreversible brain damage, coma and/or death.

Buphenyl sodium phenylbutyrate from Medicis Pharmaceutical Corp. was approved in 1996 as an adjunct to diet to treat patients with chronic UCD. In the stomach, Buphenyl is metabolized rapidly into phenylacetate, which conjugates with glutamine to form phenylacetylglutamine. Like urea, each molecule of phenylacetylglutamine contains two molecules of nitrogen and thus provides an alternative to urea for the transport and excretion of waste nitrogen.

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