Specifications
- Antibody type:Primary
- Antigen name:Hydroxymethylbilane Synthase
- Antigen symbol:HMBS
- Clonality:Polyclonal
- Conjugation:Unconjugated
- Host:Rabbit
- ImmunoChemistry:Yes
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Western blot:Yes
- Form:liquid
- Gene ID:3145
- Antigen synonyms:UPS|PORC|PBGD|PBG-D
- Storage buffer:Aqueous buffered solution containing 100ug/ml BSA, 50% glycerol and 0,09% sodium azide. Store at -20°C for 12 months.
- Concentration:1 ug/ul
- Shipping temperature:4 °C
- Purification:Purified by Protein A.
- Size:100 μg
- Pk:100 µl
Specifications
About this item
PBGD, 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.
WB(1:100-1000), IHC-P(1:100-500), IF(IHC-P)(1:50-200)
Type: Primary
Antigen: HMBS
Clonality: Polyclonal
Clone:
Conjugation: Unconjugated
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat