bs-7954R-A350

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: Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.

Storage: Store at -20°C. Aliquot into multiple vials to 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.

Conjugation: ALEXA FLUOR® 350

Excitation/ Emission: 346nm/442nm

Size: 100ul

Concentration: 1ug/ul

Applications: WB(1:300-5000)
IF(IHC-P)(1:50-200)
IF(IHC-F)(1:50-200)
IF(ICC)(1:50-200)

Predicted Molecular Weight: 36

Cross Reactive Species: Mouse

Predicted Cross Reactive Species: Human
Rat
Dog
Cow
Sheep
Horse
Rabbit

For research use only. Not intended for diagnostic or therapeutic use.