epitope corresponding to amino acids 1-265 representing full length UROS of human origin
Modification Notes
Uroporphyrinogen-III synthase (UROS), also designated hydroxymethylbilane hydrolyase, is a 265-amino acid ubiquitously expressed protein that is predominantly found in skeletal muscle, heart muscle, the caudate nucleus, and the amygdala. UROS is the fourth enzyme in the human heme biosynthetic pathway and is responsible for the conversion of hydroxymethylbilane to the cyclic tetrapyrrole, uroporphyrinogen III. UROS is a monomer involved in pophyrin biosynthesis by the Shemin pathway. Defects in the UROS gene can cause molecular lesions that lead to the autosomal recessive Gunther disease, also known as congenital erythropoietic porphyria (CEP). CEP is characterized by accumulation of uroporphyrin I in the bone marrow, peripheral blood, and other organs, often causing death early in adult life.
Key Feature
Clonality
Polyclonal
Isotype
IgG
Host Species
Rabbit
Tested Applications
WBIPIFELISA
recommended for detection of Uroporphyrinogen-III synthase (UROS) of human and, to a lesser extent, mouse and rat origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine:
Species Reactivity
HumanMouseRat
Concentration
1mg/ml
Purification
Affinity purified
Target Information
Alternative Names
epitope corresponding to amino acids 1-265 representing full length UROS of human origin
Tissue Specificity
epitope corresponding to amino acids 1-265 representing full length UROS of human origin
Western blot analysis of UROS expression in mouse thyroid (A) and mouse liver (B) tissue extracts.
Application Notes
recommended for detection of Uroporphyrinogen-III synthase (UROS) of human and, to a lesser extent, mouse and rat origin by WB, IP, IF and ELISA also reactive with additional species, including equine, canine, bovine and porcine:
Additional Information
Form
Liquid
Storage Instructions
For short-term storage, store at 4° C. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.