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Merck
CN

AV44199

Anti-ACVRL1 antibody produced in rabbit

affinity isolated antibody

Synonym(s):

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

NACRES:
NA.41
UNSPSC Code:
12352203
Conjugate:
unconjugated
Clone:
polyclonal
Application:
WB
Citations:
2

Key Documents

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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.

NP_000011

UniProt accession no.

P37023

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Gene Information

human ... ACVRL1(94)

Related Categories

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

Storage Class

10 - Combustible liquids

flash_point_f

Not applicable

flash_point_c

Not applicable

Regulatory Information

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Certificates of Analysis (COA)

Lot/Batch Number
QC14463

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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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