AV44199
Anti-ACVRL1 antibody produced in rabbit
affinity isolated antibody
别名:
Anti-ACVRLK1, Anti-ALK-1, Anti-ALK1, Anti-Activin A receptor type II-like 1, Anti-HHT, Anti-HHT2, Anti-ORW2, Anti-SKR3
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关于此项目
NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
polyclonal
Application:
WB
Citations:
2
biological source
rabbit
conjugate
unconjugated
antibody form
affinity isolated antibody
antibody product type
primary antibodies
clone
polyclonal
form
buffered aqueous solution
mol wt
55 kDa
species reactivity
human, dog
concentration
0.5 mg - 1 mg/mL
technique(s)
western blot: suitable
NCBI accession no.
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Gene Information
human ... ACVRL1(94)
Immunogen
Synthetic peptide directed towards the N terminal region of human ACVRL1
Application
Anti-ACVRL1 antibody produced in rabbit is suitable for western blotting at a concentration of 1.0μg/ml.
Biochem/physiol Actions
Activin receptor-like kinase-1 (ACVRL1; ALK1) is a type I cell surface receptor that interacts with TGF-β ligands. It influences the TGF-β-mediated transcription regulation and cell signaling. Mutations in the ALK1 gene result in hereditary hemorrhagic telangiectasia, an autosomal dominant vascular dysplasia.
Physical form
Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.
Other Notes
Synthetic peptide located within the following region: SPHCKGPTCRGAWCTVVLVREEGRHPQEHRGCGNLHRELCRGRPTEFVNH
Disclaimer
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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wgk
WGK 3
存储类别
10 - Combustible liquids
flash_point_f
Not applicable
flash_point_c
Not applicable
法规信息
常规特殊物品
此项目有
Laura Boeri et al.
Molecular syndromology, 4(3), 119-124 (2013-05-09)
Hereditary hemorrhagic telangiectasia (HHT) is an autosomal dominant vascular dysplasia. Mutations in either ENG or ACVRL1 account for around 85% of cases, and 10% are large deletions and duplications. Here we present a large novel deletion in ACVRL1 gene and
T G W Letteboer et al.
Human genetics, 116(1-2), 8-16 (2004-11-02)
Hereditary hemorrhagic telangiectasia (HHT) or Rendu-Osler-Weber disease is an autosomal dominant disorder characterized by an aberrant vascular development. The resulting vascular lesions range from smaller mucocutaneous telangiectases to large visceral arteriovenous malformations, especially in the skin, lung, gastrointestinal tract and
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