Disease Directory Congenital Disorder of Glycosylation
Metabolic

Congenital Disorder of Glycosylation

Also known as: CDG syndrome, PMM2-CDG (CDG-Ia), phosphomannomutase 2 deficiency, CDG-I and CDG-II

Prevalence

1-9 per 100,000 (Orphanet, combined CDG)

Onset

Neonatal, Infantile

Type

Autosomal recessive genetic (most types)

Gene

PMM2, MPI, ALG6, ATP6AP1

About Congenital Disorder of Glycosylation

Congenital disorders of glycosylation (CDG) are a large group of inherited metabolic disorders affecting the addition of sugar chains (glycans) to proteins and lipids. PMM2-CDG is the most common type, caused by mutations in PMM2 encoding phosphomannomutase 2, and presents with cerebellar hypoplasia, intellectual disability, and coagulopathy. Over 150 CDG subtypes exist, each caused by mutations in different glycosylation pathway genes, with widely varying severity.

Common Clinical Features

Cerebellar hypoplasia and ataxia Intellectual disability Failure to thrive Coagulopathy Pericardial effusion Retinitis pigmentosa Liver dysfunction

Clinical Trial Eligibility Tips

What to know before applying to Congenital Disorder of Glycosylation trials.

Specify the CDG subtype by gene — PMM2-CDG trials differ from SLC35C1-CDG or ATP6AP1-CDG trials

Transferrin isoelectric focusing (TIEF) or transferrin glycoform mass spectrometry is required for diagnostic confirmation and monitoring

Mannose supplementation trials are specific to MPI-CDG (CDG-Ib) — this form is treatable and molecularly distinct

CDG Consortium (NCATS) registries are actively enrolling all subtypes — registration provides access to trial matching

Patient Resources

Patient Organization

CDG Care

Visit website ↗

Natural History Registry

CDG Consortium Natural History Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:137)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Congenital Disorder of Glycosylation trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Congenital Disorder of Glycosylation. Updated daily.

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