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Merck

SAB2100095

Sigma-Aldrich

Anti-ALAS2 (ab1) antibody produced in rabbit

affinity isolated antibody

Synonym(e):

Anti-δ-Aminolevulinate synthase 2, Anti-ANH1, Anti-ASB, Anti-XLSA

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About This Item

UNSPSC-Code:
12352203
NACRES:
NA.41

Biologische Quelle

rabbit

Qualitätsniveau

Konjugat

unconjugated

Antikörperform

affinity isolated antibody

Antikörper-Produkttyp

primary antibodies

Klon

polyclonal

Form

buffered aqueous solution

Mol-Gew.

60 kDa

Speziesreaktivität

mouse, rat, pig, dog, human, bovine, horse

Konzentration

0.5 mg - 1 mg/mL

Methode(n)

western blot: suitable

UniProt-Hinterlegungsnummer

Versandbedingung

wet ice

Lagertemp.

−20°C

Posttranslationale Modifikation Target

unmodified

Angaben zum Gen

human ... ALAS2(212)

Immunogen

Synthetic peptide directed towards the N terminal region of human ALAS2

Biochem./physiol. Wirkung

ALAS2 specifies an erythroid-specific mitochondrially located enzyme. The protein catalyzes the first step in the heme biosynthetic pathway. Defects in its gene cause X-linked pyridoxine-responsive sideroblastic anemia. Alternatively spliced transcript variants encoding different isoforms have been identified.The product of this gene specifies an erythroid-specific mitochondrially located enzyme. The encoded protein catalyzes the first step in the heme biosynthetic pathway. Defects in this gene cause X-linked pyridoxine-responsive sideroblastic anemia. Alternatively spliced transcript variants encoding different isoforms have been identified.

Sequenz

Synthetic peptide located within the following region: CPILATQGPNCSQIHLKATKAGGDSPSWAKGHCPFMLSELQDGKSKIVQK

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)

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In der Dokumentenbibliothek finden Sie die Dokumentation zu den Produkten, die Sie kürzlich erworben haben.

Die Dokumentenbibliothek aufrufen

Jean-Marc Blouin et al.
Blood, 136(21), 2457-2468 (2020-07-18)
Congenital erythropoietic porphyria (CEP) is an inborn error of heme synthesis resulting from uroporphyrinogen III synthase (UROS) deficiency and the accumulation of nonphysiological porphyrin isomer I metabolites. Clinical features are heterogeneous among patients with CEP but usually combine skin photosensitivity

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