Key features and details
- Produced recombinantly (animal-free) for high batch-to-batch consistency and long term security of supply
- Rabbit monoclonal [EPR8105] to HMBS/PBGD
- Suitable for: WB
- Reacts with: Mouse, Rat, Human
Product nameAnti-HMBS/PBGD antibody [EPR8105]
See all HMBS/PBGD primary antibodies
DescriptionRabbit monoclonal [EPR8105] to HMBS/PBGD
Tested applicationsSuitable for: WBmore details
Unsuitable for: Flow Cyt,ICC/IF,IHC-P or IP
Species reactivityReacts with: Mouse, Rat, Human
corresponding to Human HMBS/PBGD aa 300-400.
Database link: P08397
- HepG2, HeLa, U937, and K562 cell lysates
This product was previously labelled as HMBS
This product is a recombinant monoclonal antibody, which offers several advantages including:
- - High batch-to-batch consistency and reproducibility
- - Improved sensitivity and specificity
- - Long-term security of supply
- - Animal-free production
Our RabMAb® technology is a patented hybridoma-based technology for making rabbit monoclonal antibodies. For details on our patents, please refer to RabMAb® patents.
Storage instructionsShipped at 4°C. Store at -20°C. Stable for 12 months at -20°C.
Dissociation constant (KD)KD = 2.94 x 10 -11 M Learn more about KD
Storage bufferpH: 7.20
Preservative: 0.01% Sodium azide
Constituents: 9% PBS, 40% Glycerol, 0.05% BSA, 50% Tissue culture supernatant
Concentration information loading...
PurityTissue culture supernatant
Immunizing Peptide (Blocking)
Our Abpromise guarantee covers the use of ab129092 in the following tested applications.
The application notes include recommended starting dilutions; optimal dilutions/concentrations should be determined by the end user.
|WB||1/1000 - 1/10000. Detects a band of approximately 42-44 kDa (predicted molecular weight: 39 kDa).Can be blocked with Recombinant Human HMBS/PBGD protein (ab123176).|
FunctionTetrapolymerization of the monopyrrole PBG into the hydroxymethylbilane pre-uroporphyrinogen in several discrete steps.
Tissue specificityIsoform 1 is ubiquitously expressed. Isoform 2 is found only in erythroid cells.
PathwayPorphyrin metabolism; protoporphyrin-IX biosynthesis; coproporphyrinogen-III from 5-aminolevulinate: step 2/4.
Involvement in diseaseDefects in HMBS are the cause of acute intermittent porphyria (AIP) [MIM:176000]. AIP is a form of porphyria. Porphyrias are inherited defects in the biosynthesis of heme, resulting in the accumulation and increased excretion of porphyrins or porphyrin precursors. They are classified as erythropoietic or hepatic, depending on whether the enzyme deficiency occurs in red blood cells or in the liver. AIP is an autosomal dominant form of hepatic porphyria characterized by acute attacks of neurological dysfunctions with abdominal pain, hypertension, tachycardia, and peripheral neuropathy. Most attacks are precipitated by drugs, alcohol, caloric deprivation, infections, or endocrine factors.
Sequence similaritiesBelongs to the HMBS family.
- Information by UniProt
- HEM3_HUMAN antibody
- HMBS antibody
- Hydroxymethylbilane synthase antibody
All lanes : Anti-HMBS/PBGD antibody [EPR8105] (ab129092) at 1/1000 dilution
Lane 1 : HepG2 cell lysates
Lane 2 : HeLa cell lysates
Lane 3 : U937 cell lysates
Lane 4 : K562 cell lysates
Lysates/proteins at 10 µg per lane.
All lanes : HRP labelled goat anti-rabbit at 1/2000 dilution
Predicted band size: 39 kDa
Observed band size: 42-44 kDa why is the actual band size different from the predicted?
Equilibrium disassociation constant (KD)
Learn more about KD
Click here to learn more about KD
ab129092 has been referenced in 2 publications.
- Chen B et al. Identification and characterization of 40 novel hydroxymethylbilane synthase mutations that cause acute intermittent porphyria. J Inherit Metab Dis 42:186-194 (2019). PubMed: 30740734
- Chen CP et al. Synergistic antitumor activity of artesunate and HDAC inhibitors through elevating heme synthesis via synergistic upregulation of ALAS1 expression. Acta Pharm Sin B 9:937-951 (2019). PubMed: 31649844