Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR15112(B)] to ALAS2/ASB - C-terminal
- Suitable for: WB, IP
- Reacts with: Mouse, Rat, Human
Product nameAnti-ALAS2/ASB antibody [EPR15112(B)] - C-terminal
See all ALAS2/ASB primary antibodies
DescriptionRabbit monoclonal [EPR15112(B)] to ALAS2/ASB - C-terminal
Tested applicationsSuitable for: WB, IPmore details
Species reactivityReacts with: Mouse, Rat, Human
within Human ALAS2/ASB aa 550 to the C-terminus. The exact sequence is proprietary.
Database link: P22557
- WB: NIH/3T3, PC-12, Rat brain, Jurkat, 293 and Human fetal liver. IP: K-562 cells.
This product was previously labelled as ALAS2
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
We are constantly working hard to ensure we provide our customers with best in class antibodies. As a result of this work we are pleased to now offer this antibody in purified format. We are in the process of updating our datasheets. The purified format is designated 'PUR' on our product labels. If you have any questions regarding this update, please contact our Scientific Support team.
Storage instructionsShipped at 4°C. Store at +4°C short term (1-2 weeks). Upon delivery aliquot. Store at -20°C long term. Avoid freeze / thaw cycle.
Storage bufferPreservative: 0.01% Sodium azide
Constituents: 59% PBS, 40% Glycerol, 0.05% BSA
Concentration information loading...
PurityProtein A purified
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.
|WB||1/1000 - 1/2000. Detects a band of approximately 65 kDa (predicted molecular weight: 65 kDa).|
|IP||1/30 - 1/50.|
Tissue specificityErythroid specific.
PathwayPorphyrin metabolism; protoporphyrin-IX biosynthesis; 5-aminolevulinate from glycine: step 1/1.
Involvement in diseaseDefects 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 similaritiesBelongs to the class-II pyridoxal-phosphate-dependent aminotransferase family.
Cellular localizationMitochondrion matrix.
- Information by UniProt
- 5 @aminolevulinate synthase erythroid specific antibody
- 5 aminolevulinate synthase 2 antibody
- 5 aminolevulinate synthase 5 aminolevulinate synthase 2 antibody
All lanes : Anti-ALAS2/ASB antibody [EPR15112(B)] - C-terminal (ab184964) at 1/1000 dilution (Purified)
Lane 1 : NIH/3T3 (Mouse embryonic fibroblast) whole cell lysates
Lane 2 : PC-12 (Rat adrenal gland pheochromocytoma) whole cell lysates
Lane 3 : Rat brain lysates
Lysates/proteins at 15 µg per lane.
All lanes : Goat Anti-Rabbit IgG H&L (HRP) (ab97051) at 1/20000 dilution
Predicted band size: 65 kDa
Observed band size: 65 kDa
ab184964 (Purified) at 1:30 dilution (2 µg) immunoprecipitating ALAS2/ASB in K-562 whole cell lysate.
Lane 1 (input): K-562 (Human chronic myelogenous leukemia lymphoblast) whole cell lysate 10 µg
Lane 2 (+): ab184964 & K-562 whole cell lysate
Lane 3 (-): Rabbit monoclonal IgG (ab172730) instead of ab184964 in K-562 whole cell lysate
For western blotting, VeriBlot for IP Detection Reagent (HRP) (ab131366) was used at 1:1000 dilution.
Blocking and diluting buffer: 5% NFDM/TBST.
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.
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
Anti-Rabbit IgG (HRP), specific to the non-reduced form of IgG at 1/1000 dilution
Predicted band size: 65 kDa
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.
ab184964 has been referenced in 6 publications.
- 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). 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). PubMed: 30765471
- Chen CP et al. Synergistic antitumor activity of artesunate and HDAC inhibitors through elevating heme synthesis via synergistic upregulation of ALAS1 expression. Acta Pharm Sin B 9:937-951 (2019). PubMed: 31649844
- Paul BT et al. Sideroflexin 4 affects Fe-S cluster biogenesis, iron metabolism, mitochondrial respiration and heme biosynthetic enzymes. Sci Rep 9:19634 (2019). PubMed: 31873120
- Liu J et al. Long non-coding RNA-dependent mechanism to regulate heme biosynthesis and erythrocyte development. Nat Commun 9:4386 (2018). PubMed: 30349036
- Yien YY et al. Mutation in human CLPX elevates levels of d-aminolevulinate synthase and protoporphyrin IX to promote erythropoietic protoporphyria. Proc Natl Acad Sci U S A 114:E8045-E8052 (2017). PubMed: 28874591