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Merck
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Key Documents

AV44199

Sigma-Aldrich

Anti-ACVRL1 antibody produced in rabbit

affinity isolated antibody

Sinónimos:

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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About This Item

UNSPSC Code:
12352203
NACRES:
NA.41

biological source

rabbit

Quality Level

100

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.

NP_000011

UniProt accession no.

P37023

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.

Sequence

Synthetic peptide located within the following region: SPHCKGPTCRGAWCTVVLVREEGRHPQEHRGCGNLHRELCRGRPTEFVNH

Physical form

Purified antibody supplied in 1x PBS buffer with 0.09% (w/v) sodium azide and 2% sucrose.

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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Storage Class

10 - Combustible liquids

wgk_germany

WGK 3

flash_point_f

Not applicable

flash_point_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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Visite la Librería de documentos

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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