• Source: Kir6.2

Kir6.2 is a major subunit of the ATP-sensitive K+ channel, a lipid-gated inward-rectifier potassium ion channel. The gene encoding the channel is called KCNJ11 and mutations in this gene are 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 G-proteins and is found associated with the sulfonylurea receptor (SUR) to constitute the ATP-sensitive K+ channel.




Pathology


Mutations in this gene are a cause of congenital hyperinsulinism (CHI), an autosomal recessive disorder characterized by unregulated insulin secretion. Defects in this gene may also contribute to autosomal dominant non-insulin-dependent diabetes mellitus type II (NIDDM).




See also


Inward-rectifier potassium ion channel
Potassium channel




References






Further reading






External links


GeneReviews/NCBI/NIH/UW entry on Familial Hyperinsulinism
GeneReviews/NCBI/NIH/UW entry on Permanent Neonatal Diabetes Mellitus
KCNJ11+protein,+human at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
SUR1+protein,+human at the U.S. National Library of Medicine Medical Subject Headings (MeSH)
This article incorporates text from the United States National Library of Medicine, which is in the public domain.

Kata Kunci Pencarian:

• Saluran kalium
• Kir6.2
• Hyperinsulinemic hypoglycemia
• ATP-sensitive potassium channel
• Inward-rectifier potassium channel
• Sulfonylurea receptor
• Permanent neonatal diabetes
• Developmental bioelectricity
• KCNJ8
• HMR 1883
• Insulin