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HMBS Rabbit anti-Human, Polyclonal, Bioss
SDP

Catalog No. 501988794
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100 μL
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50-198-8794 100 μL
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Catalog No. 50-198-8794 Supplier Bioss Supplier No. BS9068R
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Description

Rabbit Polyclonal Antibody

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 Immunofluorescence, Immunohistochemistry (Paraffin), Western Blot
Classification Polyclonal
Concentration 1 μg/mL
Conjugate Unconjugated
Formulation PBS with 1% BSA, 50% glycerol and 0.09% sodium azide; pH
Gene Hmbs
Gene Accession No. P08397
Gene Alias alternative name: porphobilinogen deaminase; BOS_14464; HEM3; hemC; HMBS; Hydroxymethylbilane synthase; PBG D; PBGD; PBG-D; PORC; Porphobilinogen deaminase; porphyria, acute; Chester type; Pre-uroporphyrinogen synthase; T25658; UPS; uroporphyrinogen I synthase; uroporphyrinogen I synthetase; URO-S; Uros1
Gene Symbols Hmbs
Host Species Rabbit
Immunogen KLH conjugated synthetic peptide derived from human HMBS/PBGD.
Purification Method Protein A
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 3145
Target Species Human
Content And Storage -20°C
Product Type Antibody
Form Liquid
Isotype IgG
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