SAB1400284
Monoclonal Anti-TTN antibody produced in mouse
clone 7D3, purified immunoglobulin, buffered aqueous solution
Synonim(y):
Anti-CMD1G, Anti-CMH9, Anti-CMPD4, Anti-DKFZp451N061, Anti-FLJ32040, Anti-LGMD2J, Anti-TMD
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About This Item
Kod UNSPSC:
12352203
NACRES:
NA.41
Polecane produkty
pochodzenie biologiczne
mouse
białko sprzężone
unconjugated
forma przeciwciała
purified immunoglobulin
rodzaj przeciwciała
primary antibodies
klon
7D3, monoclonal
Postać
buffered aqueous solution
reaktywność gatunkowa
human
metody
immunofluorescence: suitable
immunohistochemistry (formalin-fixed, paraffin-embedded sections): suitable
indirect ELISA: suitable
western blot: 1-5 μg/mL
izotyp
IgG2aκ
numer dostępu UniProt
Warunki transportu
dry ice
temp. przechowywania
−20°C
docelowa modyfikacja potranslacyjna
unmodified
informacje o genach
human ... TTN(7273)
Opis ogólny
This gene encodes a large abundant protein of striated muscle. The product of this gene is divided into two regions, a N-terminal I-band and a C-terminal A-band. The I-band, which is the elastic part of the molecule, contains two regions of tandem immunoglobulin domains on either side of a PEVK region that is rich in proline, glutamate, valine and lysine. The A-band, which is thought to act as a protein-ruler, contains a mixture of immunoglobulin and fibronectin repeats, and possesses kinase activity. A N-terminal Z-disc region and a C-terminal M-line region bind to the Z-line and M-line of the sarcomere respectively so that a single titin molecule spans half the length of a sarcomere. Titin also contains binding sites for muscle associated proteins so it serves as an adhesion template for the assembly of contractile machinery in muscle cells. It has also been identified as a structural protein for chromosomes. Considerable variability exists in the I-band, the M-line and the Z-disc regions of titin. Variability in the I-band region contributes to the differences in elasticity of different titin isoforms and, therefore, to the differences in elasticity of different muscle types. Of the many titin variants identified, five for which complete transcript information is available are described. Mutations in this gene are associated with familial hypertrophic cardiomyopathy 9 and autoantibodies to titin are produced in patients with the autoimmune disease scleroderma. (provided by RefSeq)
Immunogen
TTN (AAH58824, 1 a.a. ~ 110 a.a) partial recombinant protein with GST tag. MW of the GST tag alone is 26 KDa.
Sequence
MTTQAPTFTQPLQSVVVLEGSTATFEAHISGFPVPEVSWFRDGQVISTSTLPGVQISFSDGRAKLTIPAVTKANSGRYSLKATNGSGQATSTAELLVKAETAPPNFVQRL
Sequence
MTTQAPTFTQPLQSVVVLEGSTATFEAHISGFPVPEVSWFRDGQVISTSTLPGVQISFSDGRAKLTIPAVTKANSGRYSLKATNGSGQATSTAELLVKAETAPPNFVQRL
Postać fizyczna
Solution in phosphate buffered saline, pH 7.4
Oświadczenie o zrzeczeniu się odpowiedzialności
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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Kod klasy składowania
10 - Combustible liquids
Temperatura zapłonu (°F)
Not applicable
Temperatura zapłonu (°C)
Not applicable
Certyfikaty analizy (CoA)
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Dokumenty związane z niedawno zakupionymi produktami zostały zamieszczone w Bibliotece dokumentów.
Aurélien Perrin et al.
Annals of clinical and translational neurology, 8(9), 1906-1912 (2021-07-28)
The aim of this study was to analyze patients from two distinct families with a novel distal titinopathy phenotype associated with exactly the same CNV in the TTN gene. We used an integrated strategy combining deep phenotyping and complete molecular
Jianing Chen et al.
Frontiers in oncology, 12, 877878-877878 (2022-07-26)
Lung adenocarcinoma (LUAD) is the most common type of lung cancer, accounting for around 40%. Despite achievements in the treatment approach, the prognosis is still dismal, with overall survival of fewer than five years. Thus, novel prognostic biomarkers are needed
Meng Yu et al.
Annals of clinical and translational neurology, 6(7), 1311-1318 (2019-07-30)
We present clinical features, muscle imaging findings, and genetic characteristics of five unrelated Chinese patients with congenital titinopathy, emphasizing the diagnostic role of muscle MRI. Five patients who recessive titinopathies were recruited. All patients received muscle biopsies. Mutations were detected
Aurélien Perrin et al.
Neuromuscular disorders : NMD, 30(11), 877-887 (2020-11-01)
Next generation sequencing (NGS) has allowed the titin gene (TTN) to be identified as a major contributor to neuromuscular disorders, with high clinical heterogeneity. The mechanisms underlying the phenotypic variability and the dominant or recessive pattern of inheritance are unclear.
Aurélien Perrin et al.
Annals of clinical and translational neurology, 7(5), 846-854 (2020-04-21)
Congenital titinopathies are myopathies with variable phenotypes and inheritance modes. Here, we fully characterized, using an integrated approach (deep phenotyping, muscle morphology, mRNA and protein evaluation in muscle biopsies), two siblings with congenital multicore myopathy harboring three TTN variants predicted
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