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Invitrogen™ HMBS Polyclonal Antibody
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Catalog No. PIPA579372
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100 μg
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PIPA579372 100 μg
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Catalog No. PIPA579372 Supplier Invitrogen™ Supplier No. PA579372
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Description

Rabbit Polyclonal Antibody

Reconstitute with 0.2 mL of distilled water to yield a concentration of 500 μg/mL. Positive Control - WB: human Hela whole cell, human placenta tissue, human HepG2 whole cell, human 22RV1 whole cell, human U20S whole cell.

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.
TRUSTED_SUSTAINABILITY

Specifications

Antigen HMBS
Applications ELISA, Western Blot
Classification Polyclonal
Concentration 500 μg/mL
Conjugate Unconjugated
Formulation PBS with 4mg trehalose and 0.05mg sodium azide
Gene Hmbs
Gene Accession No. P08397, P19356, P22907
Gene Alias alternative name: porphobilinogen deaminase; HEM3; hemC; HMBS; Hydroxymethylbilane synthase; PBG D; PBGD; PBG-D; PORC; Porphobilinogen deaminase; porphyria, acute; Chester type; pre-uroporphyrinogen synthase; similar to Porphobilinogen deaminase (Hydroxymethylbilane synthase) (HMBS) (Pre-uroporphyrinogen synthase) (PBG-D); T25658; Ups; uroporphyrinogen I synthase; uroporphyrinogen I synthetase; URO-S; Uros1
Gene Symbols Hmbs
Host Species Rabbit
Immunogen E. coli-derived human HMBS recombinant protein (Position: N6-H361).
Purification Method Antigen affinity chromatography
Quantity 100 μg
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 15288, 25709, 3145
Target Species Human, Mouse, Rat
Content And Storage -20°C
Product Type Antibody
Form Lyophilized
Isotype IgG
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