HMBS Antibody

Catalog Number:

49010

Amount:

100μg

Price:

$319

Swiss-Prot No:

Swiss-Prot#:P08397

Form of Antibody:

1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.

Storage/Stability:

Immunogen:

recombinant protein

Purification:

ProA affinity purified

Specificity/Sensitivity:

Applications:

WB

Background:

PBGD (porphobilinogen deaminase), 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.

References:

appl_detail:

WB: 1:1,000-1:2,000

Datasheet PDF