catalog number :
MBS213380
products full name :
MOUSE ANTI HUMAN CYSTIC FIBROSIS TRANSMEMBRANE CONDUCTANCE REGULATOR
products short name :
CFTR
other names :
cystic fibrosis transmembrane conductance regulator; Cystic fibrosis transmembrane conductance regulator; cystic fibrosis transmembrane conductance regulator; cAMP-dependent chloride channel; channel conductance-controlling ATPase; cystic fibrosis transmembrane conductance regulator (ATP-binding cassette sub-family C, member 7); ATP-binding cassette sub-family C member 7; Channel conductance-controlling ATPase (EC:3.6.3.49); cAMP-dependent chloride channel
other gene names :
CFTR; CFTR; CF; MRP7; ABC35; ABCC7; CFTR/MRP; TNR-CFTR; dJ760C5.1; ABCC7; CFTR
uniprot entry name :
CFTR_HUMAN
specificity :
This item recognises the human cystic fibrosis transmembrane conductance regulator (CFTR). It recognises an epitope localising to the ABC transporter 2 domain, specifically to a region between amino acids 1370 to 1380.
form :
Purified. Purified IgG - liquid
concentration :
IgG concentration 1.0 mg/ml
storage stability :
Store at 4 degree C or at -20 degree C if preferred. Storage in frost-free freezers is not recommended. This product should be stored undiluted. Avoid repeated freezing and thawing as this may denature the antibody. Should this product contain a precipitate we recommend microcentrifugation before use. Shelf Life: 18 months from date of despatch.
tested application :
Immunohistology Frozen*, Immunoprecipitation (IP), Western Blot (WB)*
app notes :
Immunohistology - Frozen: Application Note: The epitope recognised by this antibody is reported to be sensitive to formaldehyde fixation and tissue processing. MyBioSource recommends the use of acetone fixation for frozen sections. . Western Blotting: Minimum Dilution: 10ug/ml; Application Note: This antibody recognises a 170kDa band under native conditions.
other info1 :
Perservative Stabilisers: 0.1% Sodium Azide (NaN3)
other info2 :
Immunogen: Fusion protein containing residues 1197-1480 of human CFTR (3). Buffer Solution: Phosphate Buffered Saline. Target Species: Human
products description :
MBS213380 recognises the human cystic fibrosis transmembrane conductance regulator (CFTR). It recognises an epitope localising to the ABC transporter 2 domain, specifically to a region between amino acids 1370 to 1380.
ncbi acc num :
NP_000483.3
ncbi gb acc num :
NM_000492.3
ncbi mol weight :
Protein Molecular Weight: 170kDA
ncbi pathways :
ABC Transporters Pathway (83035); ABC Transporters Pathway (436); ABC-family Proteins Mediated Transport Pathway (106573); AMPK Signaling Pathway (989139); AMPK Signaling Pathway (992181); Bile Secretion Pathway (193146); Bile Secretion Pathway (193095); Gastric Acid Secretion Pathway (154409); Gastric Acid Secretion Pathway (154383); Pancreatic Secretion Pathway (169306)
ncbi summary :
This gene encodes a member of the ATP-binding cassette (ABC) transporter superfamily. 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 that is involved in multi-drug resistance. The encoded protein functions as a chloride channel and controls the regulation of other transport pathways. Mutations in this gene are associated with the autosomal recessive disorders cystic fibrosis and congenital bilateral aplasia of the vas deferens. Alternatively spliced transcript variants have been described, many of which result from mutations in this gene. [provided by RefSeq, Jul 2008]
uniprot summary :
CFTR: a member of the MRP subfamily of ATP-binding cassette (ABC) transporters. ABC proteins transport various molecules across extra- and intra-cellular membranes. Functions as a chloride channel and controls the regulation of other transport pathways. Mutations have been observed in patients with the autosomal recessive disorders cystic fibrosis (CF) and congenital bilateral aplasia of the vas deferens (CBAVD). Alternative splice. Protein type: Channel, chloride; Membrane protein, integral; EC 3.6.3.49; Transporter; Transporter, ABC family; Hydrolase; Membrane protein, multi-pass. Chromosomal Location of Human Ortholog: 7q31.2. Cellular Component: recycling endosome; cell surface; protein complex; microvillus; cytoplasmic vesicle membrane; early endosome membrane; basolateral plasma membrane; apical plasma membrane; early endosome; cytoplasm; plasma membrane. Molecular Function: bicarbonate transmembrane transporter activity; protein binding; chloride channel activity; chloride channel inhibitor activity; enzyme binding; chloride transmembrane transporter activity; ATP-binding and phosphorylation-dependent chloride channel activity; channel-conductance-controlling ATPase activity; ATP binding; PDZ domain binding. Biological Process: response to drug; intracellular pH elevation; response to peptide hormone stimulus; cholesterol transport; iodide transport; water transport; transepithelial chloride transport; membrane hyperpolarization; respiratory gaseous exchange; positive regulation of vasodilation; cholesterol biosynthetic process; vasodilation; cellular response to hormone stimulus; response to estrogen stimulus; bicarbonate transport; transport; response to cytokine stimulus; transmembrane transport; sperm capacitation; lung development. Disease: Vas Deferens, Congenital Bilateral Aplasia Of; Bronchiectasis With Or Without Elevated Sweat Chloride 1; Cystic Fibrosis; Pancreatitis, Hereditary