PUS1 (Psuedouridine synthase 1) belongs to the tRNA pseudouridine synthase truA family. PUS1 functions in the conversion of uridine into pseudouridine after the nucleotide has been incorporated into RNA. It may have a functional role in tRNAs and is also thought to assist in the peptidyl transfer reaction of rRNAs. As a nucleus-resident protein, PUS1 forms a complex with RARG and the SRA1 RNA. PUS1 is widely expressed, with highest levels of expression in the brain and skeletal muscle tissues. Defects in PUS1 are a cause of myopathy with lactic acidosis and sideroblastic anemia (MLASA), also known as mitochondrial myopathy and sideroblastic anemia. MLASA is a rare autosomal recessive oxidative phosphorylation disorder specific to bone marrow and skeletal muscle. The deduced human PUS1 protein contains 348 amino acids and shares 92% sequence homology with mouse PUS1.
Key Feature
Clonality
Polyclonal
Isotype
IgG
Host Species
Rabbit
Tested Applications
WBIPIFELISA
recommended for detection of PUS1 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, bovine and porcine
Species Reactivity
HumanMouseRat
Concentration
1mg/ml
Purification
Affinity purified
Target Information
Alternative Names
epitope corresponding to amino acids 148-427 mapping at the C-terminus of PUS1 of human origin
Tissue Specificity
epitope corresponding to amino acids 148-427 mapping at the C-terminus of PUS1 of human origin
recommended for detection of PUS1 of mouse, rat and human origin by WB, IP, IF and ELISA also reactive with additional species, including equine, 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.