HMBS Polyclonal Antibody, ALEXA FLUOR® 555 Conjugated

Applications

  • WB
  • IF(IHC-P)

Reactivity

  • Human
  • Mouse
  • Rat
Overview
Catalog # bs-9068R-A555
Product Name HMBS Polyclonal Antibody, ALEXA FLUOR® 555 Conjugated
Applications WB, IF(IHC-P)
Reactivity Human, Mouse, Rat
Specifications
Conjugation ALEXA FLUOR® 555
Host Rabbit
Source KLH conjugated synthetic peptide derived from human HMBS/PBGD
Immunogen Range 21-120/361
Clonality Polyclonal
Isotype IgG
Concentration 1ug/ul
Purification Purified by Protein A.
Storage Buffer Aqueous buffered solution containing 0.01M TBS (pH 7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
Storage Condition Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.
Target
Gene ID 3145
Synonyms HEM3_HUMAN; HMBS; Hydroxymethylbilane synthase; PBG D; PBG-D; PBGD; Porphobilinogen deaminase; Pre uroporphyrinogen synthase; Pre-uroporphyrinogen synthase; UPS; Uroporphyrinogen I synthase; Uroporphyrinogen I synthetase.
Background PBGD, 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.
Application Dilution
WB 1:300-5000
IF(IHC-P) 1:50-200