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Rabbit Anti-ALAS1 Recombinant Antibody (V2-634206) (CBMAB-AP250LY)


See all ALAS1 antibodies

Summary

Host Animal
Rabbit
Specificity
Human
Clone
V2-634206
Antibody Isotype
IgG
Application
WB

Basic Information

Immunogen
A synthetic peptide corresponding to a sequence within amino acids 541-640 of human Alas1.
Host Species
Rabbit
Specificity
Human
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.
ApplicationNote
WB1:500-1:2,000

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

Format
Liquid
Buffer
PBS, pH7.3, 0.05% BSA, 50% glycerol
Preservative
0.02% sodium azide
Concentration
Batch dependent
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 1
Introduction
This gene encodes the mitochondrial enzyme which is catalyzes the rate-limiting step in heme (iron-protoporphyrin) biosynthesis. The enzyme encoded by this gene is the housekeeping enzyme; a separate gene encodes a form of the enzyme that is specific for erythroid tissue. The level of the mature encoded protein is regulated by heme: high levels of heme down-regulate the mature enzyme in mitochondria while low heme levels up-regulate. A pseudogene of this gene is located on chromosome 12. Alternative splicing results in multiple transcript variants encoding different isoforms. [provided by RefSeq, Jan 2015]
Entrez Gene ID
211
UniProt ID
P13196
Alternative Names
5'-Aminolevulinate Synthase 1; 5-Aminolevulinate Synthase 1; Aminolevulinate, Delta-, Synthase 1; Delta-Aminolevulinate Synthase 1; 5-Aminolevulinic Acid Synthase 1; Delta-ALA Synthase 1; EC 2.3.1.37; ALAS-H;
Biological Process
Erythrocyte development Source: GO_Central
Heme biosynthetic process Source: GO_Central
Hemoglobin biosynthetic process Source: GO_Central
Mitochondrion organization Source: Reactome
Protoporphyrinogen IX biosynthetic process Source: UniProtKB-UniPathway
Regulation of metabolic process Source: Reactome
Response to hypoxia Source: GO_Central
Cellular Location
Mitochondrion matrix

Lahiji, A. P., Anderson, K. E., Chan, A., Simon, A., Desnick, R. J., & Ramanujam, V. S. (2020). 5-Aminolevulinate dehydratase porphyria: Update on hepatic 5-aminolevulinic acid synthase induction and long-term response to hemin. Molecular Genetics and Metabolism, 131(4), 418-423.

Stojanovski, B. M., Hunter, G. A., Na, I., Uversky, V. N., Jiang, R. H., & Ferreira, G. C. (2019). 5-Aminolevulinate synthase catalysis: the catcher in heme biosynthesis. Molecular genetics and metabolism, 128(3), 178-189.

Badawy, A. A. B. (2019). Hypothesis: Metabolic targeting of 5-aminolevulinate synthase by tryptophan and inhibitors of heme utilisation by tryptophan 2, 3-dioxygenase as potential therapies of acute hepatic porphyrias. Medical hypotheses, 131, 109314.

Na, I., Catena, D., Kong, M. J., Ferreira, G. C., & Uversky, V. N. (2018). Anti-correlation between the dynamics of the active site loop and C-terminal tail in relation to the homodimer asymmetry of the mouse erythroid 5-aminolevulinate synthase. International journal of molecular sciences, 19(7), 1899.

Ikushiro, H., Nagami, A., Takai, T., Sawai, T., Shimeno, Y., Hori, H., ... & Yano, T. (2018). Heme-dependent inactivation of 5-aminolevulinate synthase from Caulobacter crescentus. Scientific reports, 8(1), 1-13.

Na, I., DeForte, S., Stojanovski, B. M., Ferreira, G. C., & Uversky, V. N. (2018). Molecular dynamics analysis of the structural and dynamic properties of the functionally enhanced hepta-variant of mouse 5-aminolevulinate synthase. Journal of Biomolecular Structure and Dynamics, 36(1), 152-165.

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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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