bs-9068R-A488

DATASHEET

Host: Rabbit

Target Protein: HMBS

Immunogen Range: 21-120/361

Clonality: Polyclonal

Isotype: IgG

Entrez Gene: 3145

Source: KLH conjugated synthetic peptide derived from human HMBS/PBGD

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:

PBGD, also designated hydroxymethylbilane synthase, is a cytoplasmic enzyme found in the heme synthesis pathway. PBGD belongs to the HMBS (hydroxymethylbilane synthase) family. Deficiency of PBGD causes errors in pyrrole metabolism, which in turn leads to an inherited autosomal disorder called acute intermittent porphyria (AIP). AIP is characterized by acute attacks of neurological dysfunctions with hypertension, tachycardia, peripheral neurologic disturbances, abdominal pain and excessive amounts of aminolevulinic acid and porphobilinogen in the urine.

Conjugation: ALEXA FLUOR® 488

Excitation/ Emission: 490nm/525nm

Size: 100ul

Concentration: 1ug/ul

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

Cross Reactive Species: Human
Mouse
Rat

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