Kir6.2

Kir6.2 is a major subunit of the ATP-sensitive K+ channel, an is_associated_with::inward-rectifier potassium ion channel. The is_associated_with::gene encoding the channel is called is_associated_with::KCNJ11 and mutations in this gene are associated with is_associated_with::congenital hyperinsulinism.

Structure
It is an integral membrane protein. The protein, which has a greater tendency to allow potassium to flow into a cell rather than out of a cell, is controlled by is_associated_with::G-proteins and is found associated with the is_associated_with::sulfonylurea receptor (SUR) to constitute the ATP-sensitive K+ channel.

Pathology
Mutations in this gene are a cause of familial persistent hyperinsulinemic hypoglycemia of infancy (PHHI), an autosomal recessive disorder characterized by unregulated insulin secretion. Defects in this gene may also contribute to is_associated_with::autosomal dominant is_associated_with::non-insulin-dependent diabetes mellitus type II (NIDDM).