HMBS Polyclonal Antibody, Biotin Conjugated

Applications

  • WB
  • IHC-P

Reactivity

  • Human
  • Mouse
  • Rat
Overview
Catalog # bs-9068R-Biotin
Product Name HMBS Polyclonal Antibody, Biotin Conjugated
Applications WB, IHC-P
Reactivity Human, Mouse, Rat
Specifications
Conjugation Biotin
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 for 12 months.
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
IHC-P 1:200-400