Signal Transduction Antibodies 3

Anti-KCNJ11 Antibody (CAB5765)

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SKU:
CAB5765
Product Type:
Antibody
Reactivity:
Human
Reactivity:
Mouse
Host Species:
Rabbit
Isotype:
IgG
Antibody Type:
Polyclonal Antibody
Research Area:
Signal Transduction

Description

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Antibody Name:Anti-KCNJ11 Antibody
Antibody SKU:CAB5765
Antibody Size:20uL, 50uL, 100uL
Application:WB
Reactivity:Human, Mouse
Host Species:Rabbit
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 171-390 of human KCNJ11 (NP_000516.3).
Application:WB
Recommended Dilution:WB 1:500 - 1:2000
Reactivity:Human, Mouse
Positive Samples:SKOV3, HepG2, SW620, Mouse heart
Immunogen:Recombinant fusion protein containing a sequence corresponding to amino acids 171-390 of human KCNJ11 (NP_000516.3).
Purification Method:Affinity purification
Storage Buffer:Store at -20'C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.
Isotype:IgG
Sequence:TAQA HRRA ETLI FSKH AVIA LRHG RLCF MLRV GDLR KSMI ISAT IHMQ VVRK TTSP EGEV VPLH QVDI PMEN GVGG NSIF LVAP LIIY HVID ANSP LYDL APSD LHHH QDLE IIVI LEGV VETT GITT QART SYLA DEIL WGQR FVPI VAEE DGRY SVDY SKFG NTVK VPTP LCTA RQLD EDHS LLEA LTLA SARG PLRK RSVP MAKA KPKF SISP DSLS
Gene ID:3767
Uniprot:Q14654
Cellular Location:Membrane, Multi-pass membrane protein
Calculated MW:33kDa/43kDa
Observed MW:31-43kDa
Synonyms:KCNJ11, BIR, HHF2, IKATP, KIR6.2, MODY13, PHHI, TNDM3
Background: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.
UniProt Protein Function: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.
UniProt Protein Details:

Protein type:Membrane protein, multi-pass; Membrane protein, integral; Channel, potassium

Chromosomal Location of Human Ortholog: 11p15.1

Cellular Component: ATP-sensitive potassium channel complex; integral to plasma membrane; plasma membrane; T-tubule; voltage-gated potassium channel complex

Molecular Function:ankyrin binding; ATP binding; ATP-activated inward rectifier potassium channel activity; potassium ion 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; 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

NCBI Summary: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 Code:Q14654
NCBI GenInfo Identifier:76803775
NCBI Gene ID:3767
NCBI Accession:Q14654.2
UniProt Secondary Accession:Q14654,Q2M1H7, Q58EX3, Q8IW96, B4DWI4, E9PNK0,
UniProt Related Accession:Q14654
Molecular Weight:33,263 Da
NCBI Full Name:ATP-sensitive inward rectifier potassium channel 11
NCBI Synonym Full Names:potassium voltage-gated channel subfamily J member 11
NCBI Official Symbol:KCNJ11  
NCBI Official Synonym Symbols:BIR; HHF2; PHHI; IKATP; TNDM3; KIR6.2; MODY13  
NCBI Protein Information:ATP-sensitive inward rectifier potassium channel 11
UniProt Protein Name:ATP-sensitive inward rectifier potassium channel 11
UniProt Synonym Protein Names:IKATP; Inward rectifier K(+) channel Kir6.2; Potassium channel, inwardly rectifying subfamily J member 11
Protein Family:ATP-sensitive inward rectifier potassium channel
UniProt Gene Name:KCNJ11  
UniProt Entry Name:KCJ11_HUMAN
Anti-KCNJ11 Antibody (CAB5765)Western blot analysis of extracts of various cell lines, using KCNJ11 antibody (CAB5765) at 1:1000 dilution. Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (CABS014) at 1:10000 dilution. Lysates/proteins: 25ug per lane. Blocking buffer: 3% nonfat dry milk in TBST. Detection: ECL Basic Kit (RM00020). Exposure time: 90s.
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