Disease Directory Fragile X Syndrome
Neurological

Fragile X Syndrome

Also known as: FXS, FMRP deficiency, Martin-Bell syndrome, FMR1 CGG repeat expansion

Prevalence

1-5 per 10,000 (Orphanet)

Onset

Childhood

Type

X-linked genetic (trinucleotide repeat expansion)

Gene

FMR1

About Fragile X Syndrome

Fragile X syndrome is the most common inherited cause of intellectual disability and the leading single-gene cause of autism spectrum disorder, caused by CGG trinucleotide repeat expansion in the FMR1 gene leading to silencing of FMRP protein expression. Clinical features include intellectual disability, social anxiety, repetitive behaviors, large ears, macroorchidism in males, and hyperarousal. Females are typically less severely affected. Premutation carriers (55-200 repeats) are at risk for fragile X-associated tremor/ataxia syndrome (FXTAS).

Common Clinical Features

Intellectual disability Autism spectrum features Social anxiety and avoidance Hyperactivity Macroorchidism (males) Large ears and prominent jaw Seizures (20-25%)

Clinical Trial Eligibility Tips

What to know before applying to Fragile X Syndrome trials.

Full mutation CGG repeat count (>200 repeats) confirmed by Southern blot or PCR is the diagnostic requirement for FXS trials

Premutation carriers (55-200 repeats) qualify for FXTAS trials — these are distinct populations from full mutation FXS trials

Cognitive and behavioral endpoints (VABS, SNAP-IV, ABC-C) are standard baseline and outcome assessments

FMRP-targeting trials (metformin, mGluR5 antagonists) may require washout from prior investigational therapies

Patient Resources

Patient Organization

FRAXA Research Foundation

Visit website ↗

Natural History Registry

Fragile X Online Registry with Accessible Research Database (FORWARD)

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:908)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Fragile X Syndrome trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Fragile X Syndrome. Updated daily.

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