PhenomeGene — Human Disease Gene Website SeriesRPIA

Families

RPIAdeficiency is an extremely rare genetic condition that affects how the body processes certain sugars important for brain function.

It is caused by changes in the RPIAgene. A child must inherit one non-working copy from each parent.

The condition mainly affects the brain and leads to problems with development, movement, and sometimes seizures.

Only a very small number of children have been diagnosed worldwide, so doctors are still learning about it.