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Mouse Anti-ALAS2 Recombinant Antibody (V2-634207) (CBMAB-AP251LY)


See all ALAS2 antibodies
Published Data
Mouse Anti-ALAS2 Recombinant Antibody (CAP104) in WB
Mitochondria related protein levels were evaluated by western blot in sorted cord blood derived polychromatic erythroblasts from day 16 of culture (ALAS2 (65 kDa) specific bands appear at 120 kDa, likely due to protein dimers). ...View More
Sen, T., Chen, J., & Singbrant, S. (2021). Decreased PGC1β expression results in disrupted human erythroid differentiation, impaired hemoglobinization and cell cycle exit. Scientific Reports, 11(1), 17129.
Mouse Anti-ALAS2 Recombinant Antibody (CAP104) in WB
A significant change in Alad transcription with induction of KLF1 expression.
(A) Relative mRNA levels of Alas2, Alad and Pbgd in K1-ERp cells treated with vehicle control or 4-OHT for 6 h, as determined by Q-RT-PCR. Represented mRNA levels were corrected to Hprt mRNA levels. (B) Relative RNA levels of Alad in K1-ERp cells corrected to Hprt mRNA levels, as determined by Q-RT-PCR. RNA was isolated at 0, 0.5,1,2,4 and 6 h after treatment of the cells with 4-OHT. (C) Western blot analysis of ALAS2, ALAD, PBGD proteins in K1-ERp cells treated with vehicle control or 4-OHT for 6 h. Equivalency of protein loading was verified with immunoblotting with HSC70-specific antisera *p value≤0.05. **p value ≤0.01 by Student's t-test. Data shown represents the average of at least 3 independent experiments (mean ± SEM). ...View More
Desgardin, A. D., Abramova, T., Rosanwo, T. O., Kartha, S., Shim, E. H., Jane, S. M., & Cunningham, J. M. (2012). Regulation of delta-aminolevulinic acid dehydratase by Krüppel-like factor 1.

Summary

Host Animal
Mouse
Specificity
Human, Mouse, Rat
Clone
V2-634207
Antibody Isotype
IgG1, κ
Application
IF, IHC, IP, WB, ELISA

Basic Information

Immunogen
Amino acids 61-80 near the N-terminus of ALAS-E of human origin.
Host Species
Mouse
Specificity
Human, Mouse, Rat
Antibody Isotype
IgG1, κ
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:100-1:1,000
IP1-2 µg per 100-500 µg of total protein (1 ml of cell lysate)
ELISA1:30-1:3,000
IF(ICC)1:50-1:500
IHC-P1:50-1:500

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS, 0.1% gelatin
Preservative
< 0.1% sodium azide
Concentration
0.2 mg/ml
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
5'-Aminolevulinate Synthase 2
Introduction
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]
Entrez Gene ID
Human212
Rat25748
Mouse11656
UniProt ID
HumanP22557
RatQ63147
MouseP08680
Alternative Names
5'-Aminolevulinate Synthase 2; 5-Aminolevulinate Synthase 2; Aminolevulinate, Delta-, Synthase 2; Delta-Aminolevulinate Synthase 2; 5-Aminolevulinic Acid Synthase 2; Delta-ALA Synthase 2; EC 2.3.1.37; ALAS-E; ALASE; ASB;
Biological Process
Cellular iron ion homeostasis Source: UniProtKB
Erythrocyte development Source: GO_Central
Erythrocyte differentiation Source: UniProtKB
Heme biosynthetic process Source: UniProtKB
Hemoglobin biosynthetic process Source: UniProtKB
Oxygen homeostasis Source: UniProtKB
Protoporphyrinogen IX biosynthetic process Source: UniProtKB-UniPathway
Response to hypoxia Source: UniProtKB
Cellular Location
Mitochondrion matrix
Involvement in disease
Anemia, sideroblastic, 1 (SIDBA1): A form of sideroblastic anemia that shows a variable hematologic response to pharmacologic doses of pyridoxine. 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.
Erythropoietic protoporphyria, X-linked dominant (XLDPT): The disease is caused by variants affecting the gene represented in this entry. 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). A form of porphyria. 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 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.
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For research use only. Not intended for any clinical use.

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