Analytical Data
-
Gene name
HMBS/Porphobilinogen deaminase
- Application
-
Alternative Names
PBG-D; PBGD; UPS; Hydroxymethylbilane Synthase; Uroporphyrinogen I Synthase; Porphobilinogen deaminase; Pre-uroporphyrinogen synthase
-
Species
Human
-
Source
E. coli
-
Tag
N-His
-
Purity
Greater than 95% as determined by SDS-PAGE.
-
Uniprot
P08397
-
Expression Region
Leu85~Ser337
-
Molecular Weight
34kDa
-
Endotoxin
< 1.0 EU per μg protein as determined by the LAL method.
-
Form
Freeze-dried powder
-
Buffer formulation
PBS, pH7.4, containing 0.01% SKL, 1mM DTT, 5% Trehalose and Proclin300.
-
Reconstitution
Reconstitute in ddH2O to a concentration of 0.1-0.5 mg/mL. Do not vortex.
- Customization
-
Stability Test
The thermal stability is described by the loss rate. The loss rate was determined by accelerated thermal degradation test, that is, incubate the protein at 37℃ for 48h, and no obvious degradation and precipitation were observed. The loss rate isless than 8% within the expiration date under appropriate storage condition.
-
Storage & Shelf Life
Samples are stable for up to twelve months from date of receipt at -20℃ to -80℃. Store it under sterile conditions at -20℃ to -80℃. It is recommended that the protein be aliquoted for optimal storage. Avoid repeated freeze-thaw cycles.
-
Shipping
In general, recombinant proteins are supplied as lyophilized powder and shipped at ambient temperature. For bulk packages, the proteins are provided as frozen liquid and shipped with blue ice, unless otherwise requested by the customer.
Quality inspection process
Related Products
Protein Description
HMBS (Hydroxymethylbilane Synthase) is an enzyme that plays a crucial role in the heme biosynthesis pathway, catalyzing the conversion of porphobilinogen into hydroxymethylbilane. Deficiencies in HMBS, often due to mutations in the HMBS gene, can lead to acute intermittent porphyria (AIP), a genetic disorder characterized by the accumulation of porphyrins and their precursors, resulting in severe abdominal pain, neurological symptoms, and various complications. Understanding the structure and function of recombinant HMBS/Porphobilinogen deaminase is pivotal for elucidating the mechanisms behind AIP and other porphyrias. Recombinant protein studies facilitate the exploration of enzyme kinetics, substrate specificity, and the impact of specific mutations on enzyme activity. Furthermore, producing active recombinant HMBS provides a valuable tool for drug discovery and the development of therapeutic strategies aimed at managing porphyria episodes and mitigating symptoms. Advances in recombinant DNA technology enable the generation of high-yield enzyme systems, allowing for detailed biochemical assays and the potential identification of small molecules that could be used to treat or prevent enzyme deficiencies. Overall, research into recombinant HMBS not only aids in comprehending fundamental biological processes but also paves the way for innovative treatments that address the clinical challenges posed by porphyrias.
