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Spécifications
- Antibody type:Primary
- Antigen name:Hydroxymethylbilane Synthase
- Clonality:Polyclonal
- Gene ID:3145
- Host:Rabbit
- Isotype:IgG
- Reactivity:Human,Rat,Mouse
- Antigen symbol:HMBS
- Conjugation:Alexa Fluor® 488
- ImmunoChemistry:Yes
- Size:100 µL
- Cross adsorption:No
- Form:liquid
- 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
- Cat. no.:10671-636
- Supplier No.:BS-9068R-A488
Spécifications
A propos de cet article
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® 488
Epitope:
Host: Rabbit
Isotype: IgG
Reactivity: Human, Mouse, Rat