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Rabbit Anti-phospho-Kir6.2 (Thr224)/Cy7 Conjugated antibody (bs-12181R-Cy7)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產品編號 bs-12181R-Cy7
英文名稱 Rabbit Anti-phospho-Kir6.2 (Thr224)/Cy7 Conjugated antibody
中文名稱 Cy7標記的磷酸化ATP敏感性鉀通道亞基kir6.2抗體
別    名 Kir6.2 (phospho T224); ATP sensitive inward rectifier potassium channel 11; Beta cell inward rectifier subunit; mBIR; BIR; HHF 2; HHF2; IKATP; Inward rectifier K(+) channel Kir6.2; Inwardly rectifying potassium channel KIR6.2; IRK 11; IRK11; KCNJ11; Kir 6.2; Kir6.2; MGC133230; PHHI; Potassium channel, inwardly rectifying subfamily J member 11; Potassium inwardly rectifying channel J11; TNDM 3; TNDM3.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
產品類型 磷酸化抗體 
研究領域 腫瘤  心血管  細胞生物  信號轉導  轉錄調節(jié)因子  通道蛋白  細胞膜受體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, Sheep, )
產品應用
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 44kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthesised phosphopeptide derived from human Kir6.2 around the phosphorylation site of Thr224
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產品介紹 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. [provided by RefSeq]

Function:
ATP-sensitive potassium (K(ATP)) channels are found in endocrine cells, neurons and both smooth and striated muscle, where they play an important role in controlling insulin secretion and vascular tone, and protect neurons under metabolic stress. Kir6.2 is a member of the inward rectifier potassium channel family, which is characterised by a greater tendency to allow potassium flow into the cell rather than out of it. It associates with the sulphonylurea receptor SUR1/ABCC8 to form a subfamily of K(ATP) channels that, when mutated or misregulated, are associated with forms of hyperinsulinemic hypoglycemia, neonatal diabetes, or pre-disposition to type 2 diabetes mellitus.

Subunit:
Interacts with ABCC8/SUR. Interacts with ABCC9/SUR2.

Subcellular Location:
Cell Membrane; Multi-pass membrane protein

DISEASE:
Defects in KCNJ11 are the cause of familial hyperinsulinemic hypoglycemia type 2 (HHF2) [MIM:601820]; 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) [MIM:606176]. 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) [MIM:610582]. 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.
Note=Defects in KCNJ11 may contribute to non-insulin-dependent diabetes mellitus (NIDDM), also known as diabetes mellitus type 2.

Similarity:
Belongs to the inward rectifier-type potassium channel (TC 1.A.2.1) family. KCNJ11 subfamily.

Database links:

Entrez Gene: 3767 Human

Entrez Gene: 16514 Mouse

Omim: 600937 Human

SwissProt: Q14654 Human

SwissProt: Q61743 Mouse

Unigene: 248141 Human

Unigene: 333863 Mouse



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
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