HEM3_HUMAN antibody HMBS antibody Hydroxymethylbilane synthase antibody PBG D antibody PBG-D antibody PBGD antibody PORC antibody Porphobilinogen deaminase antibody porphyria, acute; Chester type antibody Pre uroporphyrinogen synthase antibody Pre-uroporphyrinogen synthase antibody UPS antibody Uroporphyrinogen I synthase antibody Uroporphyrinogen I synthetase antibody
Product 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.
Immunogen
recombinant protein
Key Feature
Clonality
Monoclonal
Isotype
IgG
Host Species
Recombinant rabbit
Tested Applications
WB
WB:1:1,000-1:2,000
Species Reactivity
HumanMouseRat
Concentration
1mg/ml
Target Information
Alternative Names
HEM3_HUMAN antibody HMBS antibody Hydroxymethylbilane synthase antibody PBG D antibody PBG-D antibody PBGD antibody PORC antibody Porphobilinogen deaminase antibody porphyria acute Chester type antibody Pre uroporphyrinogen synthase antibody Pre-uroporphyrinogen synthase antibody UPS antibody Uroporphyrinogen I synthase antibody Uroporphyrinogen I synthetase antibody
