Product Detail
Product NameHMBS Rabbit mAb
Clone No.SC65-07
Host SpeciesRecombinant Rabbit
Clonality Monoclonal
PurificationProA affinity purified
ApplicationsWB
Species ReactivityHu, Ms, Rt
Immunogen Descrecombinant protein
ConjugateUnconjugated
Other NamesHEM3_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
Accession NoSwiss-Prot#:P08397
Uniprot
P08397
Gene ID
3145;
Calculated MW40 kDa
Formulation1*TBS (pH7.4), 1%BSA, 40%Glycerol. Preservative: 0.05% Sodium Azide.
StorageStore at -20˚C
Application Details
WB: 1:1,000-1:2,000
Western blot analysis of HMBS on different lysates using anti-HMBS antibody at 1/1,000 dilution. Positive control: Lane 1: Hela Lane 2: 293T
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.
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