MAB3480
Anti-CFTR Antibody, a.a. 1370-1380, clone M3A7
clone M3A7, Chemicon®, from mouse
Sinónimos:
CFTR
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About This Item
Código UNSPSC:
12352203
eCl@ss:
32160702
NACRES:
NA.41
Productos recomendados
origen biológico
mouse
Nivel de calidad
forma del anticuerpo
purified immunoglobulin
tipo de anticuerpo
primary antibodies
clon
M3A7, monoclonal
reactividad de especies
human
no debe reaccionar con
mouse, shark, rat
fabricante / nombre comercial
Chemicon®
técnicas
immunocytochemistry: suitable
immunoprecipitation (IP): suitable
western blot: suitable
isotipo
IgG1
Nº de acceso NCBI
Nº de acceso UniProt
Condiciones de envío
wet ice
modificación del objetivo postraduccional
unmodified
Información sobre el gen
human ... CFTR(1080)
Descripción general
CFTR (Cystic Fibrosis Transmembrane Regulator) is an integral membrane protein shown to be involved in the transport of chloride ions. It is found on the surface of the epithelial cells lining the lungs and other organs. CFTR defects are the cause of cystic fibrosis.
Cystic Fibrosis (CF) is a common lethal genetic disease caused by mutations of the gene coding for the cystic fibrosis transmembrane conductance factor, a cAMP regulated chloride channel. Approximately 70% of all CF cases share the deletion of a phenylalanine at position 508 (delta F508) which results in abnormal chloride transport. Since the CF mutation is lethal, most often by lung and liver disease, it raises the question of why this genetic disease remains as common as it is. One possible explanation is that Salmonella typhi has been shown to use CFTR to enter intestinal epithelial cells and that delta F508 heterozygote and homozygote mice showed 86% and 100% reductions in S.typhi intestinal submucosal uptake.
Cystic Fibrosis (CF) is a common lethal genetic disease caused by mutations of the gene coding for the cystic fibrosis transmembrane conductance factor, a cAMP regulated chloride channel. Approximately 70% of all CF cases share the deletion of a phenylalanine at position 508 (delta F508) which results in abnormal chloride transport. Since the CF mutation is lethal, most often by lung and liver disease, it raises the question of why this genetic disease remains as common as it is. One possible explanation is that Salmonella typhi has been shown to use CFTR to enter intestinal epithelial cells and that delta F508 heterozygote and homozygote mice showed 86% and 100% reductions in S.typhi intestinal submucosal uptake.
Especificidad
Recognizes Human cystic fibrosis transmembrane conductance regulator (CFTR). Recognizes an epitope at the C-terminal end of the second nucleotide binding in the region of residues 1370 to 1380.
Inmunógeno
Epitope: a.a. 1370-1380
Recombinant protein encoding NBF2 domain of human CFTR
Aplicación
Anti-Cystic Fibrosis Transmembrane Conductance Regulator Antibody, a.a. 1370-1380, clone M3A7 detects level of Cystic Fibrosis Transmembrane Conductance Regulator & has been published & validated for use in IC, IP & WB.
Research Category
Neuroscience
Neuroscience
Research Sub Category
Ion Channels & Transporters
Ion Channels & Transporters
Western blot: 1μg/mL, do not boil, recognizes 170kDa CTFR protein.
Immunoprecipitation
Immunofluorescence
T84 adenocarcinoma cells are a good positive control
Immunohistochemistry: 1-50μg/mL on acetone fixed frozen sections. Does not work on paraffin embedded tissue.
Optimal working dilutions must be determined by the end user.
Immunoprecipitation
Immunofluorescence
T84 adenocarcinoma cells are a good positive control
Immunohistochemistry: 1-50μg/mL on acetone fixed frozen sections. Does not work on paraffin embedded tissue.
Optimal working dilutions must be determined by the end user.
Descripción de destino
170 kDa
Forma física
Format: Purified
Protein A Purified mouse immunoglobulin in 20 mM sodium phosphate, 250 mM NaCl, pH. 7.6, with 0.1% sodium azide as a preservative.
Protein A purified
Almacenamiento y estabilidad
Maintain for 1 year at 2–8°C from date of shipment. Aliquot to avoid repeated freezing and thawing. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.
Nota de análisis
Control
T84 whole cell extract
T84 whole cell extract
Otras notas
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
Información legal
CHEMICON is a registered trademark of Merck KGaA, Darmstadt, Germany
Cláusula de descargo de responsabilidad
Unless otherwise stated in our catalog or other company documentation accompanying the product(s), our products are intended for research use only and are not to be used for any other purpose, which includes but is not limited to, unauthorized commercial uses, in vitro diagnostic uses, ex vivo or in vivo therapeutic uses or any type of consumption or application to humans or animals.
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Opcional
Referencia del producto
Descripción
Precios
Código de clase de almacenamiento
10 - Combustible liquids
Clase de riesgo para el agua (WGK)
WGK 2
Punto de inflamabilidad (°F)
Not applicable
Punto de inflamabilidad (°C)
Not applicable
Certificados de análisis (COA)
Busque Certificados de análisis (COA) introduciendo el número de lote del producto. Los números de lote se encuentran en la etiqueta del producto después de las palabras «Lot» o «Batch»
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Most F508del-CFTR is targeted to degradation at an early folding checkpoint and independently of calnexin.
Farinha, CM; Amaral, MD
Molecular and cellular biology null
Nathalie Benz et al.
PloS one, 9(2), e88964-e88964 (2014-03-04)
Cystic fibrosis (CF), the most common autosomal recessive disease in Caucasians, is due to mutations in the CFTR gene. F508del, the most frequent mutation in patients, impairs CFTR protein folding and biosynthesis. The F508del-CFTR protein is retained in the endoplasmic
Paul D W Eckford et al.
The Journal of biological chemistry, 287(44), 36639-36649 (2012-09-04)
The cystic fibrosis transmembrane conductance regulator (CFTR) acts as a channel on the apical membrane of epithelia. Disease-causing mutations in the cystic fibrosis gene can lead to CFTR protein misfolding as in the case of the F508del mutation and/or channel
Renaud Robert et al.
Molecular pharmacology, 73(2), 478-489 (2007-11-03)
The F508del mutation impairs trafficking of the cystic fibrosis transmembrane conductance regulator (CFTR) to the plasma membrane and results in a partially functional chloride channel that is retained in the endoplasmic reticulum and degraded. We recently used a novel high-throughput
Ha Won Lee et al.
PloS one, 8(3), e59992-e59992 (2013-04-05)
Low levels of insulin-like growth factor 1 (IGF-1) have been observed in the serum of cystic fibrosis (CF) patients. However, the effects of low serum IGF-1 on the cystic fibrosis transmembrane conductance regulator (CFTR), whose defective function is the primary
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