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Invitrogen
Human HMBS (aa 50-174) Control Fragment Recombinant Protein
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Product Details
RP-89538
Applications
Tested Dilution
Publications
Product Specifications
Species
Human
Expression System
E. coli
Amino acid sequence
QFEIIAMSTTGDKILDTALSKIGEKSLFTKELEHALEKNEVDLVVHSLKDLPTVLPPGFTIGAICKRENPHDAVVFHPKFVGKTLETLPEKSVVGTSSLRRAAQLQRKFPHLEFRSIRGNLNTRL
Tag
His-ABP-tag
Class
Recombinant
Type
Protein
Purity
>80% by SDS-PAGE and Coomassie blue staining
Conjugate
Form
Liquid
Concentration
≥5.0 mg/mL
Purification
purified
Storage buffer
PBS/1M urea, pH 7.4
Contains
no preservative
Storage conditions
-20°C, Avoid Freeze/Thaw Cycles
Product Specific Information
Highest antigen sequence indentity to the following orthologs: Mouse (94%), Rat (94%).
This recombinant protein control fragment may be used for blocking experiments with the corresponding antibody, PA5-52253. In IHC/ICC and WB experiments, we recommend a 100x molar excess of the protein fragment control based on the concentration and the molecular weight. Pre-incubate the antibody-protein control fragment mixture for 30 min at room temperature.
Target Information
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
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Bioinformatics
Protein Aliases: HEM3; HMBS; Hydroxymethylbilane synthase; PBG D; PBG-D; Porphobilinogen deaminase; porphyria, acute; Chester type; Pre-uroporphyrinogen synthase; uroporphyrinogen I synthase; uroporphyrinogen I synthetase
Gene Aliases: HMBS; PBG-D; PBGD; PORC; UPS
UniProt ID: (Human) P08397
Entrez Gene ID: (Human) 3145
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