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Key Documents

AV44199

Sigma-Aldrich

Anti-ACVRL1 antibody produced in rabbit

affinity isolated antibody

Synonym(e):

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

Biologische Quelle

rabbit

Qualitätsniveau

100

Konjugat

unconjugated

Antikörperform

affinity isolated antibody

Antikörper-Produkttyp

primary antibodies

Klon

polyclonal

Form

buffered aqueous solution

Mol-Gew.

55 kDa

Speziesreaktivität

human, dog

Konzentration

0.5 mg - 1 mg/mL

Methode(n)

western blot: suitable

NCBI-Hinterlegungsnummer

NP_000011

UniProt-Hinterlegungsnummer

P37023

Versandbedingung

wet ice

Lagertemp.

−20°C

Posttranslationale Modifikation Target

unmodified

Angaben zum Gen

human ... ACVRL1(94)

Immunogen

Synthetic peptide directed towards the N terminal region of human ACVRL1

Anwendung

Anti-ACVRL1 antibody produced in rabbit is suitable for western blotting at a concentration of 1.0μg/ml.

Biochem./physiol. Wirkung

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.

Sequenz

Synthetic peptide located within the following region: SPHCKGPTCRGAWCTVVLVREEGRHPQEHRGCGNLHRELCRGRPTEFVNH

Physikalische Form

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

Haftungsausschluss

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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Lagerklassenschlüssel

10 - Combustible liquids

WGK

WGK 3

Flammpunkt (°F)

Not applicable

Flammpunkt (°C)

Not applicable

Analysenzertifikate (COA)

Suchen Sie nach Analysenzertifikate (COA), indem Sie die Lot-/Chargennummer des Produkts eingeben. Lot- und Chargennummern sind auf dem Produktetikett hinter den Wörtern ‘Lot’ oder ‘Batch’ (Lot oder Charge) zu finden.

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Die Dokumentenbibliothek aufrufen

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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