antibody

Goat anti-KCNJ11/KATP Antibody

MBS421534

0.1 mg

300 USD

polyclonal

goat

nonconjugated

western blot, immunohistochemistry, enzyme immunoassay

Antibody

Goat anti-KCNJ11/KATP Antibody

KCNJ11/KATP

Goat Anti-Kcnj11 / Kir6.2 Antibody; KCNJ11; IKATP; potassium inwardly-rectifying channel, subfamily J, member 11; BIR; HHF2; KIR6.2; MGC133230; PHHI; TNDM3; ATP-sensitive inward rectifier potassium channel 11; beta-cell inward rectifier subunit; inwardly rectifying potassium channel KIR6.2; KCNJ11 antibody; IKATP antibody; potassium inwardly-rectifying channel; subfamily J; member 11 antibody; BIR antibody; HHF2 antibody; KIR6.2 antibody; MGC133230 antibody; PHHI antibody; TNDM3 antibody; ATP-sensitive inward rectifier potassium channel 11 antibody; beta-cell inward rectifier subunit antibody; inwardly rectifying potassium channel KIR6.2 antibody; potassium inwardly-rectifying channel J11 antibody; KCNJ11 / KATP /

ATP-sensitive inward rectifier potassium channel 11 isoform 1; ATP-sensitive inward rectifier potassium channel 11; ATP-sensitive inward rectifier potassium channel 11; potassium voltage-gated channel subfamily J member 11; IKATP; Inward rectifier K(+) channel Kir6.2; Potassium channel, inwardly rectifying subfamily J member 11

KCNJ11

KATP

KCNJ11; KCNJ11; BIR; HHF2; PHHI; IKATP; TNDM3; KIR6.2; MODY13

KCJ11_HUMAN

Polyclonal

Goat

Tested: Human; Expected from sequence similarity: Human

390

AEDPAKPRYRARQ

Purified from goat serum by ammonium sulphate precipitation followed by antigen affinity chromatography using the immunizing peptide

Supplied at 0.5 mg/ml in Tris saline, 0. 02% sodium azide, pH7.3 with 0.5% bovine serum albumin.

100ug specific antibody in 200ul

Aliquot and store at -20 degree C. Minimize freezing and thawing.

Peptide ELISA (EIA), Western Blot (WB), Immunohistochemistry (IHC)

Peptide ELISA: Antibody detection limit dilution 1: 32000. Immunohistochemistry: In paraffin embedded Human Pancreas shows variable staining across the islet of Langerhans. Recommended concentration, 3-5ug/ml. Western Blot: Approx 45-48kDa band observed in Human Skeletal Muscle lysates (calculated MW of 43.5kDa according to NP_000516.3). Recommended concentration: 0.1-0.3ug/ml.

Immunogen: Peptide with sequence C-AEDPAKPRYRARQ, from the internal region (near the N Terminus) of the protein sequence according to NP_000516.3. Epitope: Internal region (near the N Terminus)

Note: This antibody is expected to recognise isoform 1 (NP_000516.3) only.

62388888

NP_000516.3

NM_000525.3

33,263 Da

ABC Transporter Disorders Pathway (1268933); ABC-family Proteins Mediated Transport Pathway (1269904); ATP Sensitive Potassium Channels Pathway (1268828); Cardiac Conduction Pathway (1339115); Defective ABCC8 Can Cause Hypoglycemias And Hyperglycemias Pathway (1339134); Disease Pathway (1268854); Disorders Of Transmembrane Transporters Pathway (1268932); FOXA2 And FOXA3 Transcription Factor Networks Pathway (137911); Insulin Secretion Pathway (777534); Integration Of Energy Metabolism Pathway (1270101)

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]

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: Membrane protein, integral; Channel, potassium; Membrane protein, multi-pass. Chromosomal Location of Human Ortholog: 11p15.1. Cellular Component: ATP-sensitive potassium channel complex; axolemma; cell soma; cytosol; endoplasmic reticulum; endosome; integral to plasma membrane; mitochondrion; myelin sheath; nuclear envelope; plasma membrane; T-tubule; voltage-gated potassium channel complex. Molecular Function: ankyrin binding; ATP binding; ATP-activated inward rectifier potassium channel activity; heat shock protein binding; potassium ion binding; protein C-terminus binding; voltage-gated potassium channel activity. Biological Process: glucose metabolic process; negative regulation of insulin secretion; neurological system process; potassium ion import; regulation of insulin secretion; regulation of membrane potential; response to ATP; response to drug; response to estradiol stimulus; response to testosterone stimulus; transmembrane transport. Disease: Diabetes Mellitus, Noninsulin-dependent; Diabetes Mellitus, Permanent Neonatal; Diabetes Mellitus, Transient Neonatal, 3; Hyperinsulinemic Hypoglycemia, Familial, 2; Maturity-onset Diabetes Of The Young, Type 13

0.1 mg

300 USD