PRPF31.org
PRPF31 Patient Community logo: a DNA double helix inside a green and gold ring, with a rising sun

PRPF31 Patient Community

Our Purpose

For people and families living with PRPF31 retinitis pigmentosa (RP11). Run by patients, free and independent.

PRPF31 Explained

Retinitis pigmentosa (RP) is a group of inherited conditions in which the retina's light-sensing cells lose function over time. PRPF31 is one of the genes that cause it. RP caused by PRPF31 is also called RP11, its older name. Genetic test reports usually name the gene.

LightLensRetinaLight-sensingcells (rodsand cones)Support layer(RPE)
RP affects the retina at the back of the eye: its light-sensing cells and the support layer beneath them.

Every cell edits its genetic instructions before using them, a step called splicing. The PRPF31 protein is one of the parts that hold the cell's editing machinery together. Most of us with PRPF31 RP have one copy of the gene that doesn't work, so our cells make less of the protein than they need. The leading explanation for why the retina suffers is that it is one of the busiest tissues in the body. The rest of the body does not seem to be affected.

1The gene is copiedExonIntronExonExons are kept. Introns are cut out.2The intron loops outIntronExonExonEditing machinePRPF31The machine grips both ends of the intron.PRPF31 holds the machine together.3The exons are joinedExonExonIntronremoved4The cell makes proteinProtein
Splicing, step by step. With too little PRPF31, the editing machine works less well, and the retina seems to be hit hardest.

PRPF31 RP is rare. Even so, PRPF31 is one of the most common genetic causes of autosomal dominant RP and is probably undercounted, because some carriers never have symptoms.

Symptoms

Full Side Vision

Narrowed Side Vision

A simulated view. As side vision narrows, a crossing can look clear while cars approach unseen from the sides.

The pace varies widely, even within one family, and when symptoms start can depend partly on the type of PRPF31 change.

RP can also bring cataracts and swelling in the retina. Both can be treated, which is one reason regular check-ups matter.

Inheritance

PRPF31 RP is autosomal dominant: one changed copy of the gene is enough to put someone at risk. Each child of a person who carries it has a 50% chance of inheriting it, sons and daughters alike.

Parent withthe changeParent withoutthe changeInheritedmay or may nothave symptomsNot inheritedChanged copyWorking copy
Each child has a 50% chance of inheriting the change. Some who inherit it never develop symptoms but can still pass it on.

What makes PRPF31 unusual is that some carriers never lose any sight. This is called incomplete penetrance. In some people the healthy copy of the gene works harder and makes up most of the shortfall. Inherited differences, some near the gene and some elsewhere in our DNA, act like a dimmer on the healthy copy and help decide who is affected. So the condition can seem to skip a generation: a parent with normal sight can still pass it on.

What the retina needsHealthy copy turned upEnough PRPF31: sight keptHealthy copy turned downToo little PRPF31: vision loss
Two people can carry the identical change. What differs is how hard their healthy copy works.

Occasionally a PRPF31 change appears for the first time in someone with no family history. A genetic counselor can help a family work through who should be tested and when.

Genetic Testing

A genetic test turns a diagnosis of "RP" into "PRPF31 RP." It confirms the cause, helps relatives understand their own risk and is usually needed to take part in clinical trials.

In the US, the Foundation Fighting Blindness offers no-cost genetic testing and counseling for people with a clinical diagnosis of an inherited retinal disease, ordered by any eye care professional. Blood relatives on the affected side of the family can often get free testing for the family's PRPF31 change.

Some PRPF31 changes are large deletions or sit deep inside the gene. Not every test looks for them. When dominant RP runs in a family and no gene has been found, a genetic counselor can advise whether further testing makes sense.

Outside the US, Retina International can point to patient organizations and testing in other countries.

Our Advantage

One treatment for all

MissingsectionEarlystopSplicingerrorToo little PRPF31 proteinOne treatmentraises PRPF31
Whatever the change, the result is the same. So the fix can be the same.

Most inherited eye diseases need a treatment built for the specific gene and sometimes for the specific change in it. PRPF31 is different. Almost every PRPF31 change ends the same way: our cells make too little PRPF31 protein. So a treatment that raises the protein could help nearly all of us, whatever our exact change.

The same idea, turning up the healthy copy of a gene, is being tested in other conditions caused by too little of a protein, including a severe childhood epilepsy and an inherited optic nerve disease. Progress in any of them strengthens the case for all of them.

Research

There is currently no approved treatment for PRPF31 RP.

Research is active. Because most of us make too little PRPF31 protein, most work aims to raise the amount, by one of three routes:

Turn upthe healthy copyAdda working copyRepairthe faulty copyMorePRPF31protein
Each route works on a different copy of the gene. All three aim for the same result.

Some approaches being studied for RP in general work regardless of the gene involved, including optogenetics, which makes surviving retinal cells sensitive to light, and cell-based therapies. Some late-stage RP trials are open to people with any RP gene, PRPF31 included.

All of these approaches are investigational. None has yet been proven safe and effective for PRPF31 RP.

We don't endorse any company, treatment or trial. To see which clinical trials are recruiting, search ClinicalTrials.gov for PRPF31.

Next Steps

  1. Confirm the Gene

    Ask an eye doctor about genetic testing. In the US it can be free.

  2. See a Specialist

    Ask for a referral to a retina specialist who sees inherited retinal diseases and keep up regular check-ups.

  3. Talk with Family

    Relatives may want testing or counseling, including those with normal sight.

  4. Join the Registry

    The free My Retina Tracker Registry helps connect people with researchers and clinical trials. Anyone tested through the no-cost program is already enrolled.

  5. Find Each Other

    Join our private group, where we share experiences, questions and news.

Community

Our private Facebook group is where the PRPF31 community meets. It is for people with PRPF31, people awaiting genetic testing, parents, family members and friends. It is dedicated to PRPF31 alone. Joining takes two short questions and agreeing to the group rules.

Questions

What Is RP11?

RP11 is the older name for retinitis pigmentosa caused by changes in the PRPF31 gene. PRPF31 RP and RP11 are the same condition.

Can PRPF31 Skip a Generation?

It can seem to. Some carriers never lose any sight, but they can still pass the change on. So it may show up in a grandparent and a grandchild but not the parent in between.

Will My Children Inherit It?

Each child of a carrier has a 50% chance of inheriting the change, whether or not the parent has symptoms. A child who inherits it may or may not develop symptoms.

Does PRPF31 Affect Anything Besides the Eyes?

It doesn't seem to. The gene is active throughout the body, but PRPF31 RP appears to affect only the retina.

Is There a Treatment?

Not yet. No treatment is approved, but several are in development.

Are There Clinical Trials for PRPF31?

Yes. Search ClinicalTrials.gov for PRPF31 to see what is recruiting. Most trials require a genetic test that confirms PRPF31.

How Fast Does It Progress?

It varies widely, even within one family, and some carriers never develop symptoms. A retina specialist can track how each person's vision changes over time.

About Us

PRPF31.org is the patient community for PRPF31 retinitis pigmentosa. It was started in 2026 by people living with PRPF31 for patients, carriers and families wherever they live. It helps our community speak with researchers, doctors and foundations. We welcome links and partnerships with other patient organizations.

It is independent and self-funded: it doesn't sell anything, takes no money from companies and doesn't endorse any company, treatment or trial.

Questions or ideas? Message us on Facebook.