Thermo Fisher Scientific HMBS Polyclonal Antibody

Applications

Tested Dilution

Publications

Western Blot (WB)

0.1-0.5 µg/mL

-

Product Specifications

Species Reactivity

Human, Mouse, Rat

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

E. coli-derived human HMBS recombinant protein (Position: N6-H361). if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:HMBS,uniProtId:P08397-1,ncbiNodeId:9606,antigenRange:6-361,antigenLength:361,antigenImageFileName:PA5-79372_HMBS_P08397-1_Rabbit.svg,antigenImageFileNamePDP:PA5-79372_HMBS_P08397-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

View immunogen .st0{fill:#FFFFFF;} .st1{fill:#1E8AE7;}

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Lyophilized

Concentration

500 µg/mL

Purification

Antigen affinity chromatography

Storage buffer

PBS with 4mg trehalose

Contains

0.05mg sodium azide

Storage conditions

-20°C

Shipping conditions

Wet ice

RRID

AB_2746488

Product Specific Information

Reconstitute with 0.2 mL of distilled water to yield a concentration of 500 µg/mL.

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.