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Merck
CN

SAB2100095

Anti-ALAS2 (ab1) antibody produced in rabbit

affinity isolated antibody

Synonym(s):

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

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About This Item

NACRES:
NA.41
UNSPSC Code:
12352203

Key Documents

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Product Name

Anti-ALAS2 (ab1) antibody produced in rabbit, affinity isolated antibody

biological source

rabbit

conjugate

unconjugated

antibody form

affinity isolated antibody

antibody product type

primary antibodies

clone

polyclonal

form

buffered aqueous solution

mol wt

60 kDa

species reactivity

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

concentration

0.5 mg - 1 mg/mL

technique(s)

western blot: suitable

UniProt accession no.

P22557

shipped in

wet ice

storage temp.

−20°C

target post-translational modification

unmodified

Quality Level

100

Gene Information

human ... ALAS2(212)

Biochem/physiol Actions

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.

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.

Immunogen

Synthetic peptide directed towards the N terminal region of human ALAS2

Other Notes

Synthetic peptide located within the following region: CPILATQGPNCSQIHLKATKAGGDSPSWAKGHCPFMLSELQDGKSKIVQK

Physical form

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

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Storage Class

10 - Combustible liquids

wgk

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

Regulatory Information

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Certificates of Analysis (COA)

Lot/Batch Number
QC12303

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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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