LIM Homeobox Protein 3 (LHX3)

LHX3 encodes a member a large protein family which carry the LIM domain, a unique cysteine-rich zinc-binding domain. The encoded protein is a transcription factor that is required for pituitary development and motor neuron specification. Mutations in this gene have been associated with a syndrome of combined pituitary hormone deficiency and rigid cervical spine. Two transcripts variants encoding distinct isoforms have been identified for this gene.

The predicted protein contains 2 tandemly repeated LIM domains and a homeodomain. Alternative splicing generates 2 different mRNAs that specify the proteins Lhx3a and Lhx3b, which are 400 and 402 amino acids long, respectively.