Specifications
- Antibody type:Primary
- Antigen name:Hydroxymethylbilane Synthase
- Antigen symbol:HMBS
- Clonality:Polyclonal
- Conjugation:Alexa Fluor® 647
- Host:Rabbit
- ImmunoChemistry:Yes
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Environmentally Preferable:
- Cross adsorption:No
- 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 4°C for 12 months.
- Storage temperature:Store at –20 °C for 12 months
- Concentration:1 μg/μl
- Shipping temperature:4°C
- Purification:Purified by Protein A
- 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.
Recommended Dilutions: IF(IHC-P): 1:50-200
Type: Primary
Antigen: HMBS
Clonality: Polyclonal
Clone:
Conjugation: Alexa Fluor® 647
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat
Supplier: VWR Chemicals
Supplier: HEATHROW
Supplier: VWR Chemicals
Supplier: VWR Chemicals
Supplier: VWR Collection
Supplier: VWR Collection
Supplier: VWR Collection
Supplier: KIMBERLY CLARK
Supplier: VWR Collection
Supplier: VWR Chemicals
Supplier: Merck
Supplier: VWR Collection