HMBS antibody
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- Target See all HMBS Antibodies
- HMBS (Hydroxymethylbilane Synthase (HMBS))
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Reactivity
- Human, Mouse, Rat
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Host
- Rabbit
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Clonality
- Polyclonal
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Conjugate
- This HMBS antibody is un-conjugated
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Application
- Western Blotting (WB), Immunofluorescence (Paraffin-embedded Sections) (IF (p)), Immunohistochemistry (Paraffin-embedded Sections) (IHC (p))
- Cross-Reactivity
- Human, Mouse, Rat
- Purification
- Purified by Protein A.
- Immunogen
- KLH conjugated synthetic peptide derived from human HMBS/PBGD
- Isotype
- IgG
- Top Product
- Discover our top product HMBS Primary Antibody
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- Application Notes
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WB 1:300-5000
IHC-P 1:200-400
IF(IHC-P) 1:50-200 - Restrictions
- For Research Use only
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- Format
- Liquid
- Concentration
- 1 μg/μL
- Buffer
- 0.01M TBS( pH 7.4) with 1 % BSA, 0.02 % Proclin300 and 50 % Glycerol.
- Preservative
- ProClin
- Precaution of Use
- This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.
- Storage
- 4 °C,-20 °C
- Storage Comment
- Shipped at 4°C. Store at -20°C for one year. Avoid repeated freeze/thaw cycles.
- Expiry Date
- 12 months
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- Target
- HMBS (Hydroxymethylbilane Synthase (HMBS))
- Alternative Name
- HMBS (HMBS Products)
- Background
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Synonyms: HEM3_HUMAN, HMBS, Hydroxymethylbilane synthase, PBG D, PBG-D, PBGD, Porphobilinogen deaminase, Pre uroporphyrinogen synthase, Pre-uroporphyrinogen synthase, UPS, Uroporphyrinogen I synthase, Uroporphyrinogen I synthetase.
Background: 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.
- Gene ID
- 3145
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