ALAD Polyclonal Antibody, Cy7 Conjugated
Product Specifications
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.
Synonyms
ALAD; ALADH; ALADR; Aminolevulinate dehydratase; Aminolevulinate, delta, dehydratase; Delta aminolevulinic acid dehydratase; Delta-aminolevulinic acid dehydratase; HEM2_HUMAN; Lv; PBGS; Porphobilinogen synthase.
Gene ID
210
Swiss Prot
P13716
Cellular Locus
Cytoplasm, Nucleus, Secreted, Extracellular matrix
Host
Rabbit
Cross Reactivity
Mouse
Immunogen
151-240/330
Target
ALAD
Clonality
Polyclonal
Isotype
IgG
Conjugation
Cy7
Source
KLH conjugated synthetic peptide derived from human ALAD
Applications
WB, IF (IHC-P), IF (IHC-F), IF (ICC)
Purification
Purified by Protein A.
Excitation Emission
743nm/767nm
Concentration
1µg/µl
Dilution
WB (1:300-5000), IF (IHC-P) (1:50-200), IF (IHC-F) (1:50-200), IF (ICC) (1:50-200)
Buffer
Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
Modification
Unmodified
Storage Conditions
Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Gene ID URL
210
Curated Selection
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