Antibody data
- Antibody Data
- Antigen structure
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- Validations
- Western blot [1]
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- Product number
- BS6624 - Provider product page
- Provider
- Bioworld Technology, Inc
- Product name
- HMBS polyclonal antibody
- Antibody type
- Polyclonal
- Antigen
- Recombinant full length Human HMBS.
- 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.
- Reactivity
- Human, Mouse, Rat
- Host
- Rabbit
- Isotype
- IgG
- Vial size
- 100ul
- Concentration
- 1 mg/ml
- Storage
- Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
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Supportive validation
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- Bioworld Technology, Inc (provider)
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- Experimental details
- Western blot analysis of HMBS polyclonal antibody