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Invitrogen™ HMBS Polyclonal Antibody
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Catalog No. PIPA562230
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100 μL
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PIPA562230 100 μL
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Catalog No. PIPA562230 Supplier Invitrogen™ Supplier No. PA562230
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Description

Rabbit Polyclonal Antibody

Immunogen sequence: EFSAIILATA GLQRMGWHNR VGQILHPEEC MYAVGQGALG VEVRAKDQDI LDLVGVLHDP ETLLRCIAER AFLRHLEGGC SVPVAVHTAM KDGQLYLTGG VWSLDGSDSI QETMQATIHV PAQHEDGPED DPQLV Highest antigen sequence identity to the following orthologs: Mouse - 93%, Rat - 94%.

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 0.4 mg/mL
Conjugate Unconjugated
Formulation PBS with 40% glycerol and 0.02% sodium azide; pH 7.2
Gene Hmbs
Gene Accession No. P08397
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 Recombinant protein corresponding to Human HMBS. Recombinant protein control fragment (Product #RP-89537).
Purification Method Antigen affinity chromatography
Quantity 100 μL
Regulatory Status RUO
Primary or Secondary Primary
Gene ID (Entrez) 3145
Target Species Human
Content And Storage Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.
Product Type Antibody
Form Liquid
Isotype IgG
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