Recombinant
RabMAb

Recombinant Anti-ALAS2/ASB antibody [EPR15112(B)] - C-terminal (ab184964)

Overview

  • Product name

    Anti-ALAS2/ASB antibody [EPR15112(B)] - C-terminal
    See all ALAS2/ASB primary antibodies
  • Description

    Rabbit monoclonal [EPR15112(B)] to ALAS2/ASB - C-terminal
  • Host species

    Rabbit
  • Tested applications

    Suitable for: WB, Flow Cyt, IPmore details
  • Species reactivity

    Reacts with: Mouse, Rat, Human
  • Immunogen

    within Human ALAS2/ASB aa 550 to the C-terminus. The exact sequence is proprietary.
    Database link: P22557

  • Positive control

    • K562, Jurkat, 293 and Human fetal liver lysates; K562 cells.
  • General notes

     This product was previously labelled as ALAS2

     

    Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMab® patents.

    This product is a recombinant rabbit monoclonal antibody.

Properties

Applications

Our Abpromise guarantee covers the use of ab184964 in the following tested applications.

The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.

Application Abreviews Notes
WB 1/1000 - 1/2000. Detects a band of approximately 65 kDa (predicted molecular weight: 65 kDa).
Flow Cyt 1/100.

ab172730 - Rabbit monoclonal IgG, is suitable for use as an isotype control with this antibody.

 

IP 1/30 - 1/50.

Target

  • Tissue specificity

    Erythroid specific.
  • Pathway

    Porphyrin metabolism; protoporphyrin-IX biosynthesis; 5-aminolevulinate from glycine: step 1/1.
  • Involvement in disease

    Defects in ALAS2 are a cause of anemia sideroblastic X-linked (XLSA) [MIM:300751]. Sideroblastic anemia is characterized by anemia of varying severity, hypochromic peripheral erythrocytes, systemic iron overload secondary to chronic ineffective erythropoiesis, and the presence of bone marrow ringed sideroblasts. Sideroblasts are characterized by iron-loaded mitochondria clustered around the nucleus. XLSA shows a variable hematologic response to pharmacologic doses of pyridoxine.
    Defects in ALAS2 are the cause of erythropoietic protoporphyria X-linked dominant (XLDPT) [MIM:300752]. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. XLDPT is a form of porphyria characterized biochemically by a high proportion of zinc-protoporphyrin in erythrocytes, in which a mismatch between protoporphyrin production and the heme requirement of differentiating erythroid cells leads to overproduction of protoporphyrin in amounts sufficient to cause photosensitivity and liver disease. Note=Gain of function mutations in ALS2 are responsible for XLDPT, but they can also be a possible aggravating factor in congenital erythropoietic porphyria and other erythropoietic disorders caused by mutations in other genes (PubMed:21309041).
  • Sequence similarities

    Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family.
  • Cellular localization

    Mitochondrion matrix.
  • Information by UniProt
  • Database links

  • Alternative names

    • 5 @aminolevulinate synthase erythroid specific antibody
    • 5 aminolevulinate synthase 2 antibody
    • 5 aminolevulinate synthase 5 aminolevulinate synthase 2 antibody
    • 5 aminolevulinate synthase erythroid specific mitochondrial antibody
    • 5 aminolevulinic acid synthase 2 antibody
    • 5 aminolevulinic acid synthase antibody
    • 5-aminolevulinate synthase antibody
    • 5-aminolevulinic acid synthase 2 antibody
    • Alas 2 antibody
    • ALAS antibody
    • ALAS E antibody
    • ALAS, erythroid antibody
    • ALAS-E antibody
    • Alas2 antibody
    • ALASE antibody
    • Aminolevulinate delta synthase 2 antibody
    • Aminolevulinic acid synthase 2, erythroid antibody
    • ANH1 antibody
    • ASB antibody
    • Delta ALA synthase 2 antibody
    • Delta ALA synthetase antibody
    • Delta aminolevulinate synthase 2 antibody
    • Delta aminolevulinate synthase antibody
    • Delta-ALA synthase 2 antibody
    • Delta-aminolevulinate synthase 2 antibody
    • Erythroid specific ALAS antibody
    • erythroid-specific antibody
    • FLJ93603 antibody
    • HEM0_HUMAN antibody
    • mitochondrial antibody
    • OTTHUMP00000023388 antibody
    • OTTHUMP00000023389 antibody
    • OTTMUSP00000020679 antibody
    • RP23-338A17.1 antibody
    • SIDBA1 antibody
    • XLDPP antibody
    • XLEPP antibody
    • XLSA antibody
    see all

Images

  • All lanes : Anti-ALAS2/ASB antibody [EPR15112(B)] - C-terminal (ab184964) at 1/1000 dilution

    Lane 1 : K562 lysate
    Lane 2 : Jurkat lysate
    Lane 3 : 293 lysate

    Lysates/proteins at 20 µg per lane.

    Secondary
    All lanes : Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/1000 dilution

    Predicted band size: 65 kDa

  • Anti-ALAS2/ASB antibody [EPR15112(B)] - C-terminal (ab184964) at 1/1000 dilution + Human fetal liver at 10 µg

    Secondary
    Anti-Rabbit IgG (HRP), specific to the non-reduced form of IgG at 1/1000 dilution

    Predicted band size: 65 kDa

  • Flow cytometric analysis of K652 cells (2% paraformaldehyde-fixed) labeling ALAS2/ASB with ab184964 at 1/100 dilution (red) or a Rabbit monoclonal IgG (negative) (green) followed by Goat anti rabbit IgG (FITC) secondary at 1/150 dilution.

  • Western blot analysis of immunoprecipitation pellet from 293 cell lysate immunoprecipitated using ab184964 at 1/50 dilution. 

    Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated secondary used at a 1/1000 dilution.

References

This product has been referenced in:

  • Barman-Aksözen J  et al. Delta-aminolevulinic acid synthase 2 expression in combination with iron as modifiers of disease severity in erythropoietic protoporphyria. Mol Genet Metab N/A:N/A (2019). Read more (PubMed: 31076252) »
  • Maio N  et al. Dimeric ferrochelatase bridges ABCB7 and ABCB10 homodimers in an architecturally defined molecular complex required for heme biosynthesis. Haematologica N/A:N/A (2019). Read more (PubMed: 30765471) »
See all 4 Publications for this product

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