HMBS Recombinant Rabbit Monoclonal Antibody (8G12), Invitrogen™
Manufacturer: Thermo Scientific
Select a Size
| Pack Size | SKU | Availability | Price |
|---|---|---|---|
| Each of 1 | FEMA550030-Each-of-1 | In Stock | ₹ 37,202.00 |
FEMA550030 - Each of 1
In Stock
Quantity
1
Base Price: ₹ 37,202.00
GST (18%): ₹ 6,696.36
Total Price: ₹ 43,898.36
Antigen
HMBS
Classification
Recombinant Monoclonal
Concentration
0.67 mg/mL
Formulation
PBS with 50% glycerol and 0.02% sodium azide, pH 7.4
Gene Accession No.
P08397
Gene Symbols
Hmbs
Immunogen
A synthesized peptide derived from human HMBS
Quantity
100 μL
Primary or Secondary
Primary
Target Species
Human
Form
Liquid
Applications
ELISA, Western Blot
Clone
8G12
Conjugate
Unconjugated
Gene
Hmbs
Gene Alias
alternative name: porphobilinogen deaminase, HEM3, hemC, HMBS, Hydroxymethylbilane synthase, PBG D, PBGD, PBG-D, PORC, Porphobilinogen deaminase, porphyria, acute, Chester type, pre-uroporphyrinogen synthase, similar to Porphobilinogen deaminase (Hydroxymethylbilane synthase) (HMBS) (Pre-uroporphyrinogen synthase) (PBG-D), T25658, Ups, uroporphyrinogen I synthase, uroporphyrinogen I synthetase, URO-S, Uros1
Host Species
Rabbit
Purification Method
Affinity chromatography
Regulatory Status
RUO
Gene ID (Entrez)
3145
Content And Storage
-20°C or -80°C if preferred
Isotype
IgG
Related Products
Description
- 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.