Product nameAnti-ALAS2/ASB antibody [EPR15112(B)] - BSA and Azide free
See all ALAS2/ASB primary antibodies
DescriptionRabbit monoclonal [EPR15112(B)] to ALAS2/ASB - BSA and Azide free
Tested applicationsSuitable for: IP, WBmore details
Species reactivityReacts with: Mouse, Rat, Human
Synthetic peptide 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.
ab244239 is the carrier-free version of ab184964 This format is designed for use in antibody labeling, including fluorochromes, metal isotopes, oligonucleotides, enzymes.
Our carrier-free formats are supplied in a buffer free of BSA, sodium azide and glycerol for higher conjugation efficiency.
Use our conjugation kits for antibody conjugates that are ready-to-use in as little as 20 minutes with <1 minute hands-on-time and 100% antibody recovery: available for fluorescent dyes, HRP, biotin and gold.
Ab244239 is compatible with the Maxpar® Antibody Labeling Kit from Fluidigm.
Maxpar® is a trademark of Fluidigm Canada Inc.
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.
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.
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
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 bufferConstituent: PBS
Concentration information loading...
PurityProtein A purified
Our Abpromise guarantee covers the use of ab244239 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|IP||Use at an assay dependent concentration.|
|WB||Use at an assay dependent concentration. Detects a band of approximately 65 kDa (predicted molecular weight: 65 kDa).|
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
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.
This data was developed using the same antibody clone in a different buffer formulation containing PBS, BSA, glycerol, and sodium azide (ab184964)
ab244239 has not yet been referenced specifically in any publications.