ABCC8 Polyclonal Antibody
Ucallm_WP0909-50
Price range: $268.00 through $328.00
Product details
Background:
The protein encoded by this gene is a member of the superfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. ABC genes are divided into seven distinct subfamilies (ABC1, MDR/TAP, MRP, ALD, OABP, GCN20, White). This protein is a member of the MRP subfamily which is involved in multi-drug resistance. This protein functions as a modulator of ATP-sensitive potassium channels and insulin release. Mutations and deficiencies in this protein have been observed in patients with hyperinsulinemic hypoglycemia of infancy, an autosomal recessive disorder of unregulated and high insulin secretion. Mutations have also been associated with non-insulin-dependent diabetes mellitus type II, an autosomal dominant disease of defective insulin secretion. Alternatively spliced transcript variants have been found for
Specifications
| Size | 50μL/100μL |
| Target | ABCC8 |
| Reactivity | Human, Rat |
| Applications | WB, ELISA |
| MW(Calculated) | |
| MW(Observed) | 173kD |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-2000; ELISA 1:5000-20000 |
| Formulation | Liquid in PBS containing 50% glycerol,0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| Immunogen | Synthesized peptide derived from human protein . at AA range: 500-580 |
| Specificity | ABCC8 Polyclonal Antibody detects endogenous levels of protein. |
| Gene Name | ABCC8 HRINS SUR SUR1 |
| Protein Name | ATP-binding cassette sub-family C member 8 (Sulfonylurea receptor 1) |
| Other Name | |
| Species | Human |
| Gene ID | 6833 |
| Uniprot | Q09428, |
| Species.1 | |
| Gene ID.1 | |
| Uniprot.1 | |
| Species.2 | Rat |
| Gene ID.2 | |
| Uniprot.2 | Q09429 |
| Cellular Localization | Cell membrane; Multi-pass membrane protein . |
| Tissue Expression | Brain,Foreskin,Pancreas,Pancreatic islet, |
| Signaling_pathway | Organismal Systems >> Endocrine system >> Insulin secretion |
| Research Areas | >>ABC transporters; / >>Insulin secretion; / >>Type II diabetes mellitus |
| Function | Disease:Defects in ABCC8 are a cause of leucine-induced hypoglycemia (LIH) [MIM:240800]; also called leucine-sensitive hypoglycemia of infancy. LIH is a rare cause of hypoglycemia and is described as a condition in which symptomatic hypoglycemia is provoked by high protein feedings. Hypoglycemia is also elicited by administration of oral or intravenous infusions of a single amino acid, leucine.,Disease:Defects in ABCC8 are a cause of permanent neonatal diabetes mellitus (PNDM) [MIM:606176]; also called permanent diabetes mellitus of infancy (PDMI). PNDM is a rare form of diabetes characterized by insulin-requiring hyperglycemia that is diagnosed within the first months of life. Permanent neonatal diabetes requires lifelong therapy.,Disease:Defects in ABCC8 are the cause of familial hyperinsulinemic hypoglycemia type 1 (HHF1) [MIM:256450]; also known as persistent hyperinsulinemic hypoglycemia of infancy (PHHI) or hyperinsulinism. HHF1 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.,Disease:Defects in ABCC8 are the cause of transient neonatal diabetes mellitus type 2 (TNDM2) [MIM:610374]. Neonatal diabetes is a form of diabetes mellitus defined by the onset of mild-to-severe hyperglycemia within the first months of life. Transient neonatal diabetes remits early, with a possible relapse during adolescence.,Disease:Defects in ABCC8 may contribute to non-insulin-dependent diabetes mellitus (NIDDM), also known as diabetes mellitus type 2, in Northern European Caucasians.,Function:Putative subunit of the beta-cell ATP-sensitive potassium channel (KATP). Regulator of ATP-sensitive K(+) channels and insulin release.,similarity:Belongs to the ABC transporter family.,similarity:Belongs to the ABC transporter family. Conjugate transporter (TC 3.A.1.208) subfamily.,similarity:Contains 2 ABC transmembrane type-1 domains.,similarity:Contains 2 ABC transporter domains.,subunit:Associates with KCNJ11., |
Please login to post questions
Related products
