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This incorrect epigenotype is a result of a dysregulation in the imprinting mechanism in the parent’s germ line, often caused by microdeletions in a critical region known as the PWS-imprinting center ( The region between BP1 and BP2 on chromosome 15q11.2-q13 contains four non-imprinted genes: NIPA1, NIPA2, CYF1P1, and TUBGCP5 ( Figure 1 )

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