Disease Directory Fucosidosis
Metabolic

Fucosidosis

Also known as: FUCA1 deficiency, alpha-L-fucosidase deficiency

Prevalence

1-9 per 1,000,000 (Orphanet)

Onset

Infantile, Childhood

Type

Autosomal recessive genetic

Gene

FUCA1

About Fucosidosis

Fucosidosis is an extremely rare lysosomal storage disorder caused by mutations in the FUCA1 gene encoding alpha-L-fucosidase, an enzyme needed to break down fucose-containing glycoproteins and glycolipids. Accumulation of these substrates causes progressive neurological deterioration, coarse facial features, angiokeratoma, and recurrent infections. The disease course is variable, with two clinical forms described: an early-severe form and a later-milder form with longer survival.

Common Clinical Features

Progressive intellectual disability Coarse facial features Angiokeratoma Recurrent infections Spasticity Growth retardation Seizures

Clinical Trial Eligibility Tips

What to know before applying to Fucosidosis trials.

Alpha-L-fucosidase enzyme activity in leukocytes is the primary diagnostic and eligibility biomarker

Fucosidosis is extremely rare — contact ISMRD and Orphanet for active trial information and patient advocacy support

Natural history studies and registries are the most accessible research participation option given current lack of approved therapies

Hematopoietic stem cell transplantation has been attempted in some patients — transplant status affects future trial eligibility

Patient Resources

Patient Organization

International Mannosidosis and Related Diseases (ISMRD)

Visit website ↗

Natural History Registry

ISMRD Global Patient Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:349)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Fucosidosis trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Fucosidosis. Updated daily.

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