Typical Testing Data/Standard Curve (for reference only)
Mouse 5-aminolevulinate synthase, erythroid-specific, mitochondrial (ALAS2) ELISA Kit | ALAS2 elisa kit
Mouse 5-aminolevulinate synthase, erythroid-specific, mitochondrial (ALAS2) ELISA Kit
Typical Testing Data/Standard Curve (for reference only)
Typical Testing Data/Standard Curve (for reference only)
Principle of the Assay: ALAS2 ELISA kit applies the competitive enzyme immunoassay technique utilizing a polyclonal anti-ALAS2 antibody and an ALAS2-HRP conjugate. The assay sample and buffer are incubated together with ALAS2-HRP conjugate in pre-coated plate for one hour. After the incubation period, the wells are decanted and washed five times. The wells are then incubated with a substrate for HRP enzyme. The product of the enzyme-substrate reaction forms a blue colored complex. Finally, a stop solution is added to stop the reaction, which will then turn the solution yellow. The intensity of color is measured spectrophotometrically at 450nm in a microplate reader. The intensity of the color is inversely proportional to the ALAS2 concentration since ALAS2 from samples and ALAS2-HRP conjugate compete for the anti-ALAS2 antibody binding site. Since the number of sites is limited, as more sites are occupied by ALAS2 from the sample, fewer sites are left to bind ALAS2-HRP conjugate. A standard curve is plotted relating the intensity of the color (O.D.) to the concentration of standards. The ALAS2 concentration in each sample is interpolated from this standard curve.
NCBI and Uniprot Product Information
NCBI Description
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. [provided by RefSeq, Jul 2008]
Uniprot Description
ALAS2: Defects in ALAS2 are a cause of anemia sideroblastic X- linked (XLSA). 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). 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. 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). Belongs to the class-II pyridoxal-phosphate-dependent aminotransferase family. 3 isoforms of the human protein are produced by alternative splicing.
Protein type: Cofactor and Vitamin Metabolism - porphyrin and chlorophyll; Amino Acid Metabolism - glycine, serine and threonine; EC 2.3.1.37; Mitochondrial; Transferase
Chromosomal Location of Human Ortholog: Xp11.21
Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial inner membrane
Molecular Function: protein binding; 5-aminolevulinate synthase activity; glycine binding; coenzyme binding; pyridoxal phosphate binding
Biological Process: hemoglobin biosynthetic process; oxygen homeostasis; cellular iron ion homeostasis; porphyrin metabolic process; response to hypoxia; erythrocyte differentiation; protoporphyrinogen IX biosynthetic process; heme biosynthetic process
Disease: Anemia, Sideroblastic, X-linked; Protoporphyria, Erythropoietic, X-linked
Research Articles on ALAS2
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Product Notes
The Mouse ALAS2 alas2 (Catalog #AAA7224018) is an ELISA Kit and is intended for research purposes only. The product is available for immediate purchase. The AAA7224018 ELISA Kit recognizes Mouse ALAS2. It is sometimes possible for the material contained within the vial of "5-aminolevulinate synthase, erythroid-specific, mitochondrial (ALAS2), ELISA Kit" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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