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ZNHIT3

Involvement in disease

PEHO syndrome

PEHO

An autosomal recessive syndrome characterized by progressive encephalopathy, lack of psychomotor development, severe intellectual disability, early onset epileptic seizures, optic nerve/cerebellar atrophy, pedal edema, and early death.

None

The disease is caused by variants affecting the gene represented in this entry.

Cellular localization

Alternative names

TRIP3, ZNHIT3, Zinc finger HIT domain-containing protein 3, HNF-4a coactivator, Thyroid hormone receptor interactor 3, Thyroid receptor-interacting protein 3, TR-interacting protein 3, TRIP-3

swissprot:Q15649 entrezGene:9326 omim:604500