Anti-HMBS Mouse Monoclonal Antibody
Product Information
Downloads & Supports
Target
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HMBS
Host
Storage
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Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze/thaw cycles.
Clonality
Clone Number
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ZN144
Isotype
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IgG1
Reactivity
Application
Application Details
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IHC: 1/50-1/100
Immunogen
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Full length human recombinant protein of human HMBS (NP_000181) produced in HEK293T cell.
Molecular Weight
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-
Formulation
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PBS (PH 7.3) containing 1% BSA, 50% glycerol and 0.02% sodium azide.
Form
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Liquid
Gene ID
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3145
Uniprot ID
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P08397
Background
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This gene encodes a member of the hydroxymethylbilane synthase superfamily. The encoded protein is the third enzyme of the heme biosynthetic pathway and catalyzes the head to tail condensation of four porphobilinogen molecules into the linear hydroxymethylbilane. Mutations in this gene are associated with the autosomal dominant disease acute intermittent porphyria. Alternatively spliced transcript variants encoding different isoforms have been described. [provided by RefSeq, Jul 2008]
Research Area
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Signal Transduction
Synonyms
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UPS; PBGD; PORC; PBG-D; HMBS; Hydroxymethylbilane synthase