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TUBB3-related tubulinopathy

MedGen UID:
1002843
Concept ID:
CN322634
Disease or Syndrome
Monarch Initiative: MONDO:0100154

Definition

A tubulinopathy syndrome associated with malformations of cortical development, axon guidance defects, white matter abnormalities, and/or congenital fibrosis of the extraocular muscles (CFEOM), due to de novo or dominantly inherited variants with high penetrance. Individuals may present with variable combinations of malformations of cortical development, dysplasia of the basal ganglia, brainstem, and/or cerebellum, CFEOM, additional cranial nerve involvement, Kallmann syndrome, cyclic vomiting, peripheral neuropathy, and/or contractures. Developmental delays, intellectual disability, ocular motor apraxia, and mirror movements are also frequent features. [from MONDO]

Recent clinical studies

Etiology

Grønborg S, Kjaergaard S, Hove H, Larsen VA, Kirchhoff M
Am J Med Genet A 2015 Nov;167A(11):2731-6. Epub 2015 Jun 24 doi: 10.1002/ajmg.a.37227. PMID: 26109418

Diagnosis

Grønborg S, Kjaergaard S, Hove H, Larsen VA, Kirchhoff M
Am J Med Genet A 2015 Nov;167A(11):2731-6. Epub 2015 Jun 24 doi: 10.1002/ajmg.a.37227. PMID: 26109418

Clinical prediction guides

Grønborg S, Kjaergaard S, Hove H, Larsen VA, Kirchhoff M
Am J Med Genet A 2015 Nov;167A(11):2731-6. Epub 2015 Jun 24 doi: 10.1002/ajmg.a.37227. PMID: 26109418

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