Human Cystic Fibrosis Transmembrane Conductance Regulator ELISA kit

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Product name Human Cystic Fibrosis Transmembrane Conductance Regulator ELISA kit
Short Description Human ELISA Kit to CFTR.
Description The kit is a sandwich enzyme immunoassay for in vitro quantitative measurement of CFTR in Human serum, plasma and other biological fluids.
Applications ELISA
Dilution range 0.156--10ng/mL
Protein Name CFTR
Storage Instruction Store at 4°C for 6 months.
Note FOR RESEARCH USE ONLY (RUO) .
Validated Application ELISA
Reactivity Human
Gene ID 1080
Gene Symbol CFTR
Molecular Weight 168.1 kDa
Database Links HGNC:1884
OMIM:219700
Reactome:R-HSA-382556
Alternative Names Cystic Fibrosis Transmembrane Conductance Regulator, Cftr, Atp-Binding Cassette Sub-Family C Member 7, Channel Conductance-Controlling Atpase, Camp-Dependent Chloride Channel
Function Epithelial ion channel that plays an important role in the regulation of epithelial ion and water transport and fluid homeostasis. Mediates the transport of chloride ions across the cell membrane. Channel activity is coupled to ATP hydrolysis. The ion channel is also permeable to HCO (3-) ; selectivity depends on the extracellular chloride concentration. Exerts its function also by modulating the activity of other ion channels and transporters. Plays an important role in airway fluid homeostasis. Contributes to the regulation of the pH and the ion content of the airway surface fluid layer and thereby plays an important role in defense against pathogens. Modulates the activity of the epithelial sodium channel (ENaC) complex, in part by regulating the cell surface expression of the ENaC complex. Inhibits the activity of the ENaC channel containing subunits SCNN1A, SCNN1B and SCNN1G. Inhibits the activity of the ENaC channel containing subunits SCNN1D, SCNN1B and SCNN1G, but not of the ENaC channel containing subunits SCNN1A, SCNN1B and SCNN1G. May regulate bicarbonate secretion and salvage in epithelial cells by regulating the transporter SLC4A7. Can inhibit the chloride channel activity of ANO1. Plays a role in the chloride and bicarbonate homeostasis during sperm epididymal maturation and capacitation.
Post-translational Modifications N-glycosylated.; Phosphorylated; cAMP treatment promotes phosphorylation and activates the channel. Dephosphorylation decreases the ATPase activity (in vitro) . Phosphorylation at PKA sites activates the channel. Phosphorylation at PKC sites enhances the response to phosphorylation by PKA. Phosphorylated by AMPK; this inhibits channel activity.; Ubiquitinated, leading to its degradation in the lysosome. Deubiquitination by USP10 in early endosomes enhances its endocytic recycling to the cell membrane. Ubiquitinated by RNF185 during ER stress.
Cellular Localization Apical Cell Membrane, Multi-Pass Membrane Protein, Early Endosome Membrane, Cell Membrane, Recycling Endosome Membrane, Endoplasmic Reticulum Membrane, Nucleus, Note=The Channel Is Internalized From The Cell Surface Into An Endosomal Recycling Compartment, From Where It Is Recycled To The Cell Membrane (Pubmed:17462998, Pubmed:19398555, Pubmed:20008117) , In The Oviduct And Bronchus, Detected On The Apical Side Of Epithelial Cells, But Not Associated With Cilia (Pubmed:22207244) , In Sertoli Cells, A Processed Product Is Detected In The Nucleus (By Similarity) , Er Stress Induces Gorasp2-Mediated Unconventional (Er/Golgi-Independent) Trafficking Of Core-Glycosylated Cftr To Cell Membrane (Pubmed:21884936)
Tissue Specificity Serum, Plasma, Biological Fluids
Swiss-Prot Key CFTR_HUMAN
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