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HMBS Rabbit anti-Human, Mouse, Rat, Polyclonal, Proteintech

Rabbit Polyclonal Antibody

Brand:  Proteintech 14797-1-AP-150UL

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Product Code. 16893614

  • 4796.50 NOK / 150µL

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Description

Description

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

Specifications

HMBS
Polyclonal
Unconjugated
HMBS
HMBS, hydroxymethylbilane synthase, PBG D, PBGD, Porphobilinogen deaminase, Pre uroporphyrinogen synthase, UPS
Rabbit
Antigen Affinity Chromatography
RUO
15288, 25709, 3145
-20°C
Liquid
Immunocytochemistry, Immunofluorescence, Immunoprecipitation, Western Blot
0.2 mg/mL
PBS with 50% glycerol and 0.02% sodium azide; pH 7.3
P08397, P19356, P22907
Hmbs
HMBS Fusion Protein Ag6509
150 μL
Primary
Human, Mouse, Rat
Antibody
IgG
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