Thermo Fisher Scientific HMBS Recombinant Rabbit Monoclonal Antibody (8G12)

Applications

Tested Dilution

Publications

Western Blot (WB)

1:500-1:2,000

-

ELISA (ELISA)

Assay-dependent

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG

Expression System

HEK293 cells

Class

Recombinant Monoclonal

Type

Antibody

Clone

8G12

Immunogen

A synthesized peptide derived from human HMBS

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

0.67 mg/mL

Purification

Affinity chromatography

Storage buffer

PBS, pH 7.4, with 50% glycerol

Contains

0.02% sodium azide

Storage conditions

-20°C or -80°C if preferred

Shipping conditions

Wet ice

RRID

AB_3092855

Target Information

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.

For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.