Thermo Fisher Scientific HMBS Monoclonal Antibody (OTI3F4)

Applications

Tested Dilution

Publications

Western Blot (WB)

1:2,000

-

Immunohistochemistry (Paraffin) (IHC (P))

1:150

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Mouse / IgG1

Class

Monoclonal

Type

Antibody

Clone

OTI3F4

Immunogen

Full length human recombinant protein of HMBS produced in HEK293T cell if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:HMBS,uniProtId:P08397-1,ncbiNodeId:9606,antigenRange:1-361,antigenLength:361,antigenImageFileName:MA5-26545_HMBS_P08397-1_House_mouse.svg,antigenImageFileNamePDP:MA5-26545_HMBS_P08397-1_House_mouse_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

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Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

1 mg/mL

Purification

Affinity Chromatography

Storage buffer

PBS, pH 7.3, with 1% BSA, 50% glycerol

Contains

0.02% sodium azide

Storage conditions

-20° C, Avoid Freeze/Thaw Cycles

Shipping conditions

Ambient (domestic); Wet ice (international)

RRID

AB_2724460

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.