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Invitrogen™ HMBS Polyclonal Antibody
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Catalog No. PIPA5102482
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Quantity:
100 μL
Catalog No. Quantity
PIPA5102482 100 μL
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Catalog No. PIPA5102482

Supplier: Invitrogen™ PA5102482

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Description

Rabbit Polyclonal Antibody

Antibody detects endogenous levels of total HMBS.

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 Immunohistochemistry (Paraffin), Western Blot, Immunocytochemistry
Classification Polyclonal
Concentration 1 mg/mL
Conjugate Unconjugated
Formulation PBS with 50% glycerol and 0.02% sodium azide; pH 7.4
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 A synthesized peptide derived from human HMBS(Accession P08397), corresponding to amino acid residues K140-T190.
Purification Method Affinity Chromatography
Quantity 100 μL
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 Liquid
Isotype IgG
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