MODY Type 7

Definition

Heterozygous variants in the KLF11 gene disrupt a transcription factor involved in the transcriptional regulation of the insulin gene in pancreatic beta cells; loss-of-function variants reduce insulin secretory capacity. MODY7 is a very rare subtype; clinical diagnostic criteria include diabetes onset before age 25 and an autosomal dominant family history.

Gene/region analyzed

KLF11 - Whole Gene

Method

Sequence analysis

Accepted sample types

EDTA blood, AF, CVS

Inheritance

Hereditary, autosomal dominant.

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