ALAS1抗体

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     The image is immunohistochemistry of paraffin-embedded Human breast cancer tissue using P07808(ALAS1 Antibody) at dilution 1/15. (Original magnification: ×200)    

  
  

ALAS1 (5-aminolevulinate synthase 1) is a mitochondrial enzyme encoded by the ALAS1 gene, primarily involved in the first and rate-limiting step of heme biosynthesis. It catalyzes the condensation of glycine and succinyl-CoA to form 5-aminolevulinic acid (ALA), a critical precursor in heme production. Unlike its erythroid-specific isoform ALAS2. ALAS1 is ubiquitously expressed, particularly in the liver, and plays a central role in maintaining cellular heme homeostasis. Heme, an essential cofactor for cytochromes and hemoproteins, regulates ALAS1 through feedback inhibition, while its expression is transcriptionally controlled by factors like oxidative stress, hormones, and metabolic cues.

Antibodies targeting ALAS1 are widely used in research to study heme metabolism disorders, hepatic dysfunction, and diseases linked to mitochondrial anomalies. For instance, ALAS1 dysregulation is implicated in porphyrias, sideroblastic anemia, and lead poisoning, where enzyme inhibition disrupts heme synthesis. ALAS1 antibodies enable detection and quantification of the enzyme in tissues or cell lysates via techniques like Western blotting, immunohistochemistry, and immunofluorescence. They also aid in exploring ALAS1's role in drug-induced liver injury, as certain medications (e.g., barbiturates) induce ALAS1 expression, potentially exacerbating porphyria symptoms. Additionally, these antibodies contribute to investigations of metabolic pathways, mitochondrial dynamics, and cross-talk between heme synthesis and cellular stress responses, making them valuable tools in both basic and clinical research.