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HMBS Mouse anti-Human, Clone: OTI3E2, lyophilized, TrueMAB™

Product Code. 17667121
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Quantity:
100 μg
Unit Size:
100µg
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17667121 100 μg 100µg
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Product Code. 17667121 Supplier Origene Technologies Supplier No. CF802728

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Description

Mouse Monoclonal Antibody

Reconstitute with PBS (pH 7.3) and recommend to perform another round of desalting process using Product No. 7KMWCO

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.

Specifications

Antigen HMBS
Applications Western Blot
Classification Monoclonal
Clone OTI3E2
Conjugate Unconjugated
Formulation PBS with 8% trehalose and no preservative; pH 7.3
Gene HMBS
Gene Accession No. P08397
Gene Alias PBG-D, PBGD, PORC, UPS
Gene Symbols HMBS
Host Species Mouse
Immunogen Full length human recombit protein of human HMBS produced in HEK293T cell.
Purification Method Affinity Chromatography
Quantity 100 μg
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 3145
Target Species Human
Content And Storage -20° C, Avoid Freeze/Thaw Cycles
Product Type Antibody
Form Lyophilized
Isotype IgG1
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