This webpage contains legacy information. The product is either no longer available from the supplier or has been delisted at Labome.
product summary
company name :
Invitrogen
other brands :
NeoMarkers, Lab Vision, Endogen, Pierce, BioSource International, Zymed Laboratories, Caltag, Molecular Probes, Research Genetics, Life Technologies, Applied Biosystems, GIBCO BRL, ABgene, Dynal, Affinity BioReagents, Nunc, Invitrogen, NatuTec, Oxoid, Richard-Allan Scientific, Arcturus, Perseptive Biosystems, Proxeon, eBioscience
product type :
antibody
product name :
HMBS Polyclonal Antibody
catalog :
PA5-44169
quantity :
100 uL
price :
US 481.00
clonality :
polyclonal
host :
domestic rabbit
conjugate :
nonconjugated
reactivity :
human
application :
western blot
product information
Product Type :
Antibody
Product Name :
HMBS Polyclonal Antibody
Catalog # :
PA5-44169
Quantity :
100 uL
Price :
US 481.00
Clonality :
Polyclonal
Purity :
Affinity Chromatography
Host :
Rabbit
Reactivity :
Human
Applications :
Western Blot: 0.2-1 ug/mL
Species :
Human
Isotype :
IgG
Storage :
-20 C, Avoid Freeze/Thaw Cycles
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 :
Synthetic peptide directed towards the N-terminal of human HMBS (aa 18-67).
Format :
Liquid
Applications w/Dilutions :
Western Blot: 0.2-1 ug/mL
Aliases :
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
company information
Invitrogen
Thermo Fisher Scientific
81 Wyman Street
Waltham, MA USA 02451
https://www.thermofisher.com
800-678-5599
headquarters: USA