Western Blot (WB)
(Western blot analysis of extracts of various cell lines, using KCNJ11 antibody.)
Rabbit KCNJ11 Polyclonal Antibody | anti-KCNJ11 antibody
KCNJ11 (ATP-Sensitive Inward Rectifier Potassium Channel 11, BIR, HHF2, PHHI, IKATP, TNDM3, KIR6.2) (FITC)
WB: 1:500-1:2000
Applications are based on unconjugated antibody.
Western Blot (WB)
(Western blot analysis of extracts of various cell lines, using KCNJ11 antibody.)
Western Blot (WB)
(Western blot analysis of extracts of various cell lines, using KCNJ11 antibody.)
NCBI and Uniprot Product Information
NCBI Description
Potassium channels are present in most mammalian cells, where they participate in a wide range of physiologic responses. The protein encoded by this gene is an integral membrane protein and inward-rectifier type potassium channel. The encoded 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. 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 autosomal dominant non-insulin-dependent diabetes mellitus type II (NIDDM), transient neonatal diabetes mellitus type 3 (TNDM3), and permanent neonatal diabetes mellitus (PNDM). Multiple alternatively spliced transcript variants that encode different protein isoforms have been described for this gene. [provided by RefSeq, Oct 2009]
Uniprot Description
Kir6.2: 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. Subunit of ATP-sensitive potassium channels (KATP). Can form cardiac and smooth muscle-type KATP channels with ABCC9. KCNJ11 forms the channel pore while ABCC9 is required for activation and regulation. Defects in KCNJ11 are the cause of familial hyperinsulinemic hypoglycemia type 2 (HHF2); also known as persistent hyperinsulinemic hypoglycemia of infancy (PPHI) or congenital hyperinsulinism. HHF 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. Defects in KCNJ11 are a cause of diabetes mellitus permanent neonatal (PNDM). PNDM is a rare form of diabetes distinct from childhood-onset autoimmune diabetes mellitus type 1. It is characterized by insulin-requiring hyperglycemia that is diagnosed within the first months of life. Permanent neonatal diabetes requires lifelong therapy. Defects in KCNJ11 are the cause of transient neonatal diabetes mellitus type 3 (TNDM3). Neonatal diabetes mellitus, defined as insulin-requiring hyperglycemia within the first month of life, is a rare entity. In about half of the neonates, diabetes is transient and resolves at a median age of 3 months, whereas the rest have a permanent form of diabetes. In a significant number of patients with transient neonatal diabetes mellitus, diabetes type 2 appears later in life. The onset and severity of TNDM3 is variable with childhood-onset diabetes, gestational diabetes or adult-onset diabetes described. Defects in KCNJ11 may contribute to non-insulin- dependent diabetes mellitus (NIDDM), also known as diabetes mellitus type 2. Belongs to the inward rectifier-type potassium channel (TC 1.A.2.1) family. KCNJ11 subfamily.
Protein type: Channel, potassium; Membrane protein, multi-pass; Membrane protein, integral
Chromosomal Location of Human Ortholog: 11p15.1
Cellular Component: voltage-gated potassium channel complex; mitochondrion; endoplasmic reticulum; integral to plasma membrane; T-tubule; ATP-sensitive potassium channel complex; nuclear envelope; axolemma; cytosol; cell soma; plasma membrane; endosome; myelin sheath
Molecular Function: potassium ion binding; protein C-terminus binding; voltage-gated potassium channel activity; G-protein activated inward rectifier potassium channel activity; heat shock protein binding; ATP-activated inward rectifier potassium channel activity; ankyrin binding; ATP binding
Biological Process: response to drug; synaptic transmission; regulation of membrane potential; potassium ion import; energy reserve metabolic process; glucose metabolic process; response to testosterone stimulus; regulation of insulin secretion; response to ATP; response to estradiol stimulus; neurological system process; negative regulation of insulin secretion
Disease: Maturity-onset Diabetes Of The Young, Type 13; Hyperinsulinemic Hypoglycemia, Familial, 2; Diabetes Mellitus, Transient Neonatal, 3; Diabetes Mellitus, Permanent Neonatal; Diabetes Mellitus, Noninsulin-dependent
Research Articles on KCNJ11
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Product Notes
The KCNJ11 kcnj11 (Catalog #AAA6423661) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The KCNJ11 (ATP-Sensitive Inward Rectifier Potassium Channel 11, BIR, HHF2, PHHI, IKATP, TNDM3, KIR6.2) (FITC) reacts with Human, Mouse, Rat and may cross-react with other species as described in the data sheet. AAA Biotech's KCNJ11 can be used in a range of immunoassay formats including, but not limited to, Immunohistochemistry (IHC), Western Blot (WB). IHC: 1:50-1:200 WB: 1:500-1:2000 Applications are based on unconjugated antibody. Researchers should empirically determine the suitability of the KCNJ11 kcnj11 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "KCNJ11, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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