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TBR1

Function

Transcriptional repressor involved in multiple aspects of cortical development, including neuronal migration, laminar and areal identity, and axonal projection (PubMed:25232744, PubMed:30250039). As transcriptional repressor of FEZF2, it blocks the formation of the corticospinal (CS) tract from layer 6 projection neurons, thereby restricting the origin of CS axons specifically to layer 5 neurons (By similarity).

Involvement in disease

Intellectual developmental disorder with autism and speech delay

IDDAS

An autosomal dominant neurodevelopmental disorder characterized by varying degrees of intellectual disability, autism spectrum disorder, and language deficits.

None

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

Tissue Specificity

Brain.

Cellular localization

Alternative names

T-box brain protein 1, T-brain-1, TBR-1, TES-56, TBR1

swissprot:Q16650 entrezGene:10716 omim:604616