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KCNQ1OT1

MIM.604115 11p15.5

Pathology

- microdeletion of LIT1 in familial Beckwith-Wiedemann Syndrome (15372379)

  • When inherited maternally, the deletion causes BWS with silencing of p57(KIP2), indicating deletion of an element important for the regulation of p57(KIP2) expression. (15372379)
  • When inherited paternally, there is no phenotype, suggesting that the LIT1 RNA itself is not necessary for normal development in humans. (15372379)