추천 제품
생물학적 소스
mouse
Quality Level
항체 형태
purified immunoglobulin
항체 생산 유형
primary antibodies
클론
M3A7, monoclonal
종 반응성
human
반응하면 안 됨
mouse, shark, rat
제조업체/상표
Chemicon®
기술
immunocytochemistry: suitable
immunoprecipitation (IP): suitable
western blot: suitable
동형
IgG1
NCBI 수납 번호
UniProt 수납 번호
배송 상태
wet ice
타겟 번역 후 변형
unmodified
유전자 정보
human ... CFTR(1080)
일반 설명
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.
특이성
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.
면역원
Epitope: a.a. 1370-1380
Recombinant protein encoding NBF2 domain of human CFTR
애플리케이션
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.
표적 설명
170 kDa
물리적 형태
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
저장 및 안정성
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.
분석 메모
Control
T84 whole cell extract
T84 whole cell extract
기타 정보
Concentration: Please refer to the Certificate of Analysis for the lot-specific concentration.
법적 정보
CHEMICON is a registered trademark of Merck KGaA, Darmstadt, Germany
면책조항
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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Storage Class Code
10 - Combustible liquids
WGK
WGK 2
Flash Point (°F)
Not applicable
Flash Point (°C)
Not applicable
시험 성적서(COA)
제품의 로트/배치 번호를 입력하여 시험 성적서(COA)을 검색하십시오. 로트 및 배치 번호는 제품 라벨에 있는 ‘로트’ 또는 ‘배치’라는 용어 뒤에서 찾을 수 있습니다.
C terminus of nucleotide binding domain 1 contains critical features for cystic fibrosis transmembrane conductance regulator trafficking and activation.
Billet, A; Melin, P; Jollivet, M; Mornon, JP; Callebaut, I; Becq, F
The Journal of Biological Chemistry null
Establishment and characterization of a novel polarized MDCK epithelial cellular model for CFTR studies
Mendes, Filipa, et al
Cellular Physiology and Biochemistry, 16, 281-290 (2005)
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
Unusually common cystic fibrosis mutation in Portugal encodes a misprocessed protein.
Mendes, Filipa, et al.
Biochemical and biophysical research communications, 311, 665-671 (2003)
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