Species: | Rabbit |
Applications: | WB IHC IF |
Immunogen Range: | KLH conjugated synthetic peptide derived from human ALAD |
Clonality: | Polyclonal Antibody |
Isotype: | IgG |
GENE ID: | 210 |
Swiss Prot: | |
Synonyms: | ALAD, ALADH, ALADR, Aminolevulinate dehydratase, Aminolevulinate, delta, dehydratase, Delta aminolevulinic acid dehydratase, Delta-aminolevulinic acid dehydratase, HEM2_HUMAN, Lv, PBGS, Porphobilinogen synthase. |
Purification: | Purified by Protein A. |
Storage: | Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0.09% sodium azide. Store at -20℃ for 12 months |
Background: | Catalyzes an early step in the biosynthesis of tetrapyrroles. Binds two molecules of 5-aminolevulinate per subunit, each at a distinct site, and catalyzes their condensation to form porphobilinogen.Involvement in disease:Defects in ALAD are the cause of acute hepatic porphyria (AHP). AHP is 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. AHP is characterized by attacks of gastrointestinal disturbances, abdominal colic, paralysis, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors. |
Caculated MW: | / |
Observed MW: | Refer to Figures |
Applications: |
WB 1:100-1:1000 IHC 1:100-1:500 IF 1:50-1:200 |
Reacitivity: | Human, Mouse, Rat |