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PAX9

Function

Transcription factor required for normal development of thymus, parathyroid glands, ultimobranchial bodies, teeth, skeletal elements of skull and larynx as well as distal limbs.

Involvement in disease

Tooth agenesis, selective, 3

STHAG3

A form of selective tooth agenesis, a common anomaly characterized by the congenital absence of one or more teeth. Selective tooth agenesis without associated systemic disorders has sometimes been divided into 2 types: oligodontia, defined as agenesis of 6 or more permanent teeth, and hypodontia, defined as agenesis of less than 6 teeth. The number in both cases does not include absence of third molars (wisdom teeth).

None

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

Cellular localization

Alternative names

Paired box protein Pax-9, PAX9

swissprot:P55771 entrezGene:5083 omim:167416