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AB114246

Recombinant Human CFTR protein

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Recombinant Human CFTR protein is a Human Fragment protein, in the 1381 to 1480 aa range, expressed in Wheat germ, suitable for SDS-PAGE, ELISA, WB.

View Alternative Names

ABCC7, CFTR, Cystic fibrosis transmembrane conductance regulator, ATP-binding cassette sub-family C member 7, Channel conductance-controlling ATPase, cAMP-dependent chloride channel

1 Images
SDS-PAGE - Recombinant Human CFTR protein (AB114246)
  • SDS-PAGE

Unknown

SDS-PAGE - Recombinant Human CFTR protein (AB114246)

12.5% SDS-PAGE showing ab114246 at approximately 36.63kDa stained with Coomassie Blue.

Key facts

Expression system

Wheat germ

Tags

Tag free

Applications

ELISA, SDS-PAGE, WB

applications

Biologically active

No

Accession

P13569

Animal free

No

Carrier free

No

Species

Human

Storage buffer

pH: 8 Constituents: 0.79% Tris HCl, 0.3% Glutathione

storage-buffer

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Reactivity data

{ "title": "Reactivity Data", "filters": { "stats": ["", "Reactivity", "Dilution Info", "Notes"] }, "values": { "SDS-PAGE": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "ELISA": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p></p>" }, "WB": { "reactivity":"TESTED_AND_REACTS", "dilution-info":"", "notes":"<p>(Recombinant protein).</p>" } } }
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Sequence info

[{"sequence":"YQIIRRTLKQAFADCTVILCEHRIEAMLECQQFLVIEENKVRQYDSIQKLLNERSLFRQAISPSDRVKLFPHRNSSKCKSKPQIAALKEETEEEVQDTRL","proteinLength":"Fragment","predictedMolecularWeight":"36.63 kDa","actualMolecularWeight":null,"aminoAcidEnd":1480,"aminoAcidStart":1381,"nature":"Recombinant","expressionSystem":"Wheat germ","accessionNumber":"P13569","tags":[]}]
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Properties and storage information

Shipped at conditions
Dry Ice
Appropriate short-term storage conditions
-80°C
Appropriate long-term storage conditions
-80°C
Aliquoting information
Upon delivery aliquot
Storage information
Avoid freeze / thaw cycle
False
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Supplementary information

This supplementary information is collated from multiple sources and compiled automatically.

The CFTR protein also known as the cystic fibrosis transmembrane conductance regulator acts as a channel that facilitates the transport of chloride ions across cell membranes. This protein plays an essential role in maintaining ion balance and hydration in various tissues. CFTR is a large glycoprotein with a molecular mass of approximately 170 kDa. It is expressed in the epithelial cells of organs such as the lungs pancreas and intestines. Its location and function underpin its significance in cellular activities and interactions.
Biological function summary

CFTR impacts the regulation of epithelial fluid movement and mucus consistency. It integrates into the cellular membrane interacting with other protein channels to form a regulatory complex. The CFTR protein's ability to transport chloride ions aids in maintaining the osmotic gradients required for proper mucus hydration. This helps prevent the build-up of thick mucus particularly in the respiratory system where it can impact breathing and infection resistance.

Pathways

The CFTR protein functions critically in electrolyte transport pathways that are connected to the regulation of ion channels and cellular signaling processes. It interacts closely with other protein channels and transporters such as ENaC forming part of the epithelial sodium channels pathway. This pathway contributes significantly to the balance of salt and water transport across epithelial surfaces. Another related pathway involves its interaction with the cAMP-dependent protein kinase (PKA) which phosphorylates CFTR modulating its activity and stability in response to cellular signals.

CFTR mutations are directly linked to cystic fibrosis a genetic condition that affects the lungs and digestive system. In cystic fibrosis dysfunctional CFTR proteins lead to the production of thick and sticky mucus resulting in respiratory and digestive complications. CFTR is also associated with congenital bilateral absence of the vas deferens (CBAVD) often related to male infertility. In both conditions protein interactions and chloride transport disruption serve as critical influences on disease progression and symptom manifestation.
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Specifications

Form

Liquid

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General info

Function

Epithelial ion channel that plays an important role in the regulation of epithelial ion and water transport and fluid homeostasis (PubMed : 26823428). Mediates the transport of chloride ions across the cell membrane (PubMed : 10792060, PubMed : 11524016, PubMed : 11707463, PubMed : 12519745, PubMed : 12529365, PubMed : 12588899, PubMed : 12727866, PubMed : 15010471, PubMed : 17036051, PubMed : 1712898, PubMed : 17182731, PubMed : 19398555, PubMed : 19621064, PubMed : 22178883, PubMed : 25330774, PubMed : 26846474, PubMed : 28087700, PubMed : 8910473, PubMed : 9804160). Possesses an intrinsic ATPase activity and utilizes ATP to gate its channel; the passive flow of anions through the channel is gated by cycles of ATP binding and hydrolysis by the ATP-binding domains (PubMed : 11524016, PubMed : 15284228, PubMed : 26627831, PubMed : 8910473). The ion channel is also permeable to HCO(3)(-); selectivity depends on the extracellular chloride concentration (PubMed : 15010471, PubMed : 19019741). In vitro, mediates ATP-dependent glutathione flux (PubMed : 12727866). Exerts its function also by modulating the activity of other ion channels and transporters (PubMed : 12403779, PubMed : 22121115, PubMed : 22178883, PubMed : 27941075). Plays an important role in airway fluid homeostasis (PubMed : 16645176, PubMed : 19621064, PubMed : 26823428). 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 (PubMed : 14668433, PubMed : 16645176, PubMed : 26823428). Modulates the activity of the epithelial sodium channel (ENaC) complex, in part by regulating the cell surface expression of the ENaC complex (PubMed : 17182731, PubMed : 17434346, PubMed : 27941075). Inhibits the activity of the ENaC channel containing subunits SCNN1A, SCNN1B and SCNN1G (PubMed : 17182731). 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 (PubMed : 17182731, PubMed : 27941075). May regulate bicarbonate secretion and salvage in epithelial cells by regulating the transporter SLC4A7 (PubMed : 12403779). Can inhibit the chloride channel activity of ANO1 (PubMed : 22178883). Plays a role in the chloride and bicarbonate homeostasis during sperm epididymal maturation and capacitation (PubMed : 19923167, PubMed : 27714810).

Sequence similarities

Belongs to the ABC transporter superfamily. ABCC family. CFTR transporter (TC 3.A.1.202) subfamily.

Post-translational modifications

N-glycosylated.. Phosphorylated; cAMP treatment promotes phosphorylation and activates the channel (PubMed:12588899, PubMed:17036051, PubMed:8910473). Dephosphorylation decreases the ATPase activity (in vitro) (PubMed:8910473). Phosphorylation at PKA sites activates the channel (PubMed:10792060, PubMed:12519745, PubMed:12588899, PubMed:25330774). Phosphorylation at PKC sites enhances the response to phosphorylation by PKA (PubMed:12588899). Phosphorylated by AMPK; this inhibits channel activity (PubMed:12519745).. Ubiquitinated, leading to its degradation in the lysosome (PubMed:19398555, PubMed:23818989). Deubiquitination by USP10 in early endosomes enhances its endocytic recycling to the cell membrane (PubMed:19398555). Ubiquitinated by RNF185 during ER stress (PubMed:24019521). Ubiquitinated by MARCHF2 (PubMed:23818989).

Subcellular localisation

Early endosome membrane

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Product protocols

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Target data

Epithelial ion channel that plays an important role in the regulation of epithelial ion and water transport and fluid homeostasis (PubMed : 26823428). Mediates the transport of chloride ions across the cell membrane (PubMed : 10792060, PubMed : 11524016, PubMed : 11707463, PubMed : 12519745, PubMed : 12529365, PubMed : 12588899, PubMed : 12727866, PubMed : 15010471, PubMed : 17036051, PubMed : 1712898, PubMed : 17182731, PubMed : 19398555, PubMed : 19621064, PubMed : 22178883, PubMed : 25330774, PubMed : 26846474, PubMed : 28087700, PubMed : 8910473, PubMed : 9804160). Possesses an intrinsic ATPase activity and utilizes ATP to gate its channel; the passive flow of anions through the channel is gated by cycles of ATP binding and hydrolysis by the ATP-binding domains (PubMed : 11524016, PubMed : 15284228, PubMed : 26627831, PubMed : 8910473). The ion channel is also permeable to HCO(3)(-); selectivity depends on the extracellular chloride concentration (PubMed : 15010471, PubMed : 19019741). In vitro, mediates ATP-dependent glutathione flux (PubMed : 12727866). Exerts its function also by modulating the activity of other ion channels and transporters (PubMed : 12403779, PubMed : 22121115, PubMed : 22178883, PubMed : 27941075). Plays an important role in airway fluid homeostasis (PubMed : 16645176, PubMed : 19621064, PubMed : 26823428). 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 (PubMed : 14668433, PubMed : 16645176, PubMed : 26823428). Modulates the activity of the epithelial sodium channel (ENaC) complex, in part by regulating the cell surface expression of the ENaC complex (PubMed : 17182731, PubMed : 17434346, PubMed : 27941075). Inhibits the activity of the ENaC channel containing subunits SCNN1A, SCNN1B and SCNN1G (PubMed : 17182731). 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 (PubMed : 17182731, PubMed : 27941075). May regulate bicarbonate secretion and salvage in epithelial cells by regulating the transporter SLC4A7 (PubMed : 12403779). Can inhibit the chloride channel activity of ANO1 (PubMed : 22178883). Plays a role in the chloride and bicarbonate homeostasis during sperm epididymal maturation and capacitation (PubMed : 19923167, PubMed : 27714810).
See full target information CFTR
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