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Mendeliome v2.496 MSX2 upstream regulatory region Sarah Milton Marked Region: MSX2 upstream regulatory region as ready
Mendeliome v2.496 MSX2 upstream regulatory region Sarah Milton Region: msx2 upstream regulatory region has been classified as Amber List (Moderate Evidence).
Mendeliome v2.496 MSX2 upstream regulatory region Sarah Milton Classified Region: MSX2 upstream regulatory region as Amber List (moderate evidence)
Mendeliome v2.496 MSX2 upstream regulatory region Sarah Milton Region: msx2 upstream regulatory region has been classified as Amber List (Moderate Evidence).
Mendeliome v2.495 MSX2 upstream regulatory region Sarah Milton Region: MSX2 upstream regulatory region was added
Region: MSX2 upstream regulatory region was added to Mendeliome. Sources: Literature
regulatory region tags were added to Region: MSX2 upstream regulatory region.
Mode of inheritance for Region: MSX2 upstream regulatory region was set to MONOALLELIC, autosomal or pseudoautosomal, NOT imprinted
Publications for Region: MSX2 upstream regulatory region were set to 22717651; 42609732
Phenotypes for Region: MSX2 upstream regulatory region were set to Parietal foramina 1 MIM#168500; Parietal foramina with cleidocranial dysplasia MIM#168550
Review for Region: MSX2 upstream regulatory region was set to AMBER
Added comment: MSX2 encodes a homeobox transcription factor that is known to have a role in osteogenic growth.

Sequence variants in MSX2 result in Craniosynostosis 2, MIM#604757, Parietal foramina 1 MIM#168500.

PMID: 22717651 summarises 2 unrelated individuals with duplications approx 10-70kb upstream of MSX2 presenting with cleidocranial dysplasia. Mechanism of disease postulated to be overexpression of MSX2, authors report enhancers were underlying some of the duplicated region.

PMID: 42609732 summarised 3 individuals from 2 unrelated families with parietal foramina with microduplications upstream of MSX2.

Mechanism remains somewhat undefined with rare reports.

Note: minimum duplicated region has been used for this entry
Sources: Literature