DATASHEET
Host:
Rabbit
Target Protein:
ALAD
Immunogen Range:
151-240/330
Clonality:
Polyclonal
Isotype:
IgG
Entrez Gene:
210
Swiss Prot:
P13716
Source:
KLH conjugated synthetic peptide derived from human ALAD
Purification:
Purified by Protein A.
Storage Buffer:
0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Storage:
Shipped at 4C. Store at -20C for one year. Avoid repeated freeze/thaw cycles.
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.
VALIDATION IMAGES
Mouse liver lysates probed with ALAD Polyclonal Antibody, Unconjugated (bs-7954R) at 1:1000 dilution and 4˚C overnight incubation. Followed by conjugated secondary antibody incubation at 1:20000 for 60 min at 37˚C.
25 ug total protein per lane of various lysates (see on figure) probed with ALAD polyclonal antibody, unconjugated (bs-7954R) at 1:1000 dilution and 4°C overnight incubation. Followed by conjugated secondary antibody incubation at r.t. for 60 min.