Thermo Fisher Scientific HMBS Polyclonal Antibody

Applications

Tested Dilution

Publications

Western Blot (WB)

0.04-0.4 µg/mL

-

Immunohistochemistry (Paraffin) (IHC (P))

1:500-1:1,000

-

Immunocytochemistry (ICC/IF)

0.25-2 µg/mL

-

Product Specifications

Species Reactivity

Human

Host/Isotype

Rabbit / IgG

Class

Polyclonal

Type

Antibody

Immunogen

Recombinant protein corresponding to Human HMBS. Recombinant protein control fragment (Product #RP-89537). if (typeof window.$mangular === undefined || !window.$mangular) { window.$mangular = {}; } $mangular.antigenJson = \[{targetFamily:HMBS,uniProtId:P08397-1,ncbiNodeId:9606,antigenRange:182-316,antigenLength:361,antigenImageFileName:PA5-62230_HMBS_P08397-1_Rabbit.svg,antigenImageFileNamePDP:PA5-62230_HMBS_P08397-1_Rabbit_PDP.jpeg,sortOrder:1}\]; $mangular.isB2BCMGT = false; $mangular.isEpitopesModalImageMultiSizeEnabled = true;

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

Conjugate

Unconjugated Unconjugated Unconjugated

Form

Liquid

Concentration

0.4 mg/mL

Purification

Antigen affinity chromatography

Storage buffer

PBS, pH 7.2, with 40% glycerol

Contains

0.02% sodium azide

Storage conditions

Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.

Shipping conditions

Wet ice

RRID

AB_2642471

Product Specific Information

Immunogen sequence: EFSAIILATA GLQRMGWHNR VGQILHPEEC MYAVGQGALG VEVRAKDQDI LDLVGVLHDP ETLLRCIAER AFLRHLEGGC SVPVAVHTAM KDGQLYLTGG VWSLDGSDSI QETMQATIHV PAQHEDGPED DPQLV

Highest antigen sequence identity to the following orthologs: Mouse - 93%, Rat - 94%.

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.