Potassium Inwardly-Rectifying Channel, Subfamily J, Member 11 (KCNJ11) (C-Term) antibody

Antigen: Potassium Inwardly-Rectifying Channel, Subfamily J, Member 11 (KCNJ11)

Synonyms: BIR, HHF2, PHHI, IKATP, TNDM3, KIR6.2, MGC133230, KCNJ11, Kir6.2, kir6.2

Epitope: C-Term

Clonality: Polyclonal

Host: Rabbit

Application: Immunohistochemistry (IHC), Western Blotting (WB)

Catalog no.: ABIN350386

Additional Information

Alternative name: KCNJ11 (Kir6.2) (c-terminal region)

Swiss-Prot: Q14654

Immunogen: A synthetic peptide from the c-terminal region of human KCNJ11 (Kir6.2) conjugated to an immunogenic carrier protein was used as the immunogen. The antigen shares 94% identity with rat and mouse sequence.

Format: Lyophilized

Description: FUNCTION: This receptor is controlled by G proteins. Inward rectifier potassium channels are characterized by a greater tendency to allow potassium to flow into the cell rather than out of it. Their voltage dependence is regulated by the concentration of extracellular potassium; as external potassium is raised, the voltage range of the channel opening shifts to more positive voltages. The inward rectification is mainly due to the blockage of outward current by internal magnesium. Can be blocked by extracellular barium. Defects in KCNJ11 are the cause of familial hyperinsulinemic hypoglycemia type 2 (HHF2); also known as persistent hyperinsulinemic hypoglycemia of infancy (PPHI) or hyperinsulinism. HHF2 is the most common cause of persistent hypoglycemia in infancy and is due to defective negative feedback regulation of insulin secretion by low glucose levels. It causes nesidioblastosis, a diffuse abnormality of the pancreas in which there is extensive, often disorganized formation of new islets. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur. Subcellular location: Membrane, Multi-pass membrane protein. Also known as: ATP-sensitive inward rectifier potassium channel 11, Potassium channel, inwardly rectifying subfamily J member 11, Inward rectifier K(+) channel Kir6.2, IKATP.

Specificity: Appears to be specific for KCNJ11.

Application Details

Application Notes: IHC, WB. A dilution of 1 : 300 to 1 : 2000 is recommended. The optimal dilution should be determined by the end user. Not yet tested in other applications.

Purity: whole serum

Storage: Maintain the lyophilised/reconstituted antibodies frozen at -20°C for long term storage and refrigerated at 2-8°C for a shorter term. When reconstituting, glycerol (1:1) may be added for an additional stability. Avoid freeze and thaw cycles.

Restrictions: For Research Use only

Publications

Publications: Inagaki, Gonoi, Clement et al.: "Reconstitution of IKATP: an inward rectifier subunit plus the sulfonylurea receptor." in: Science (New York, N.Y.), Vol. 270, Issue 5239, pp. 1166-70, 1996 (PubMed).

Sakura, Wat, Horton et al.: "Sequence variations in the human Kir6.2 gene, a subunit of the beta-cell ATP-sensitive K-channel: no association with NIDDM in while Caucasian subjects or evidence of abnormal function when expressed in vitro." in: Diabetologia, Vol. 39, Issue 10, pp. 1233-6, 1997 (PubMed).

Meissner, Beinbrech, Mayatepek: "Congenital hyperinsulinism: molecular basis of a heterogeneous disease." in: Human mutation, Vol. 13, Issue 5, pp. 351-61, 1999 (PubMed).

Halushka, Fan, Bentley et al.: "Patterns of single-nucleotide polymorphisms in candidate genes for blood-pressure homeostasis." in: Nature genetics, Vol. 22, Issue 3, pp. 239-47, 1999 (PubMed).

Stanik, Gasperikova, Paskova et al.: "Prevalence of permanent neonatal diabetes in Slovakia and successful replacement of insulin with sulfonylurea therapy in KCNJ11 and ABCC8 mutation carriers." in: The Journal of clinical endocrinology and metabolism, Vol. 92, Issue 4, pp. 1276-82, 2007 (PubMed).