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The antibody against USB1 was raised in rabbit using the Recombinant Human U6 snRNA phosphodiesterase protein (1-90AA) as the immunogen. This antibody exists as a non-conjugated isotype IgG, purified by protein G with a purity greater than 95%. This antibody has been validated on ELISA, IHC.
The antibody against USB1 was raised in rabbit using the Recombinant Human U6 snRNA phosphodiesterase protein (1-90AA) as the immunogen. This antibody exists as a non-conjugated isotype IgG, purified by protein G with a purity greater than 95%. This antibody has been validated on ELISA, IHC.
$299.00
| Cat.No | ADC-04952A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | USB1 |
| Target Synonyms | C16orf57 antibody; Chromosome 16 open reading frame 57 antibody; CP057_HUMAN antibody; FLJ13154 antibody; UPF0406 protein C16orf57 antibody | Form | Liquid |
| Species Reactivity | Human | Isotype | IgG |
| Storage Buffer | 0.01M PBS, 0.03% Proclin 300; Constituents: 50% Glycerol, PH 7.4 | Purification Method | >95%, Protein G purified |
| Conjugate | Non-conjugated | Application | ELISA, IHC |
| Storage | Upon receipt |
| Immunogen Description | Recombinant Human U6 snRNA phosphodiesterase protein (1-90AA) | Target Species | Human |
|---|---|---|---|
| Immunogen Sequence | Complete sequences for the immunogen, target protein, and peptides are available upon request. | Uniprot ID | Q9BQ65 |
Uniprot Id
Q9BQ65
Target Species
Human
Target Name
USB1
Target Full Name
U6 snRNA phosphodiesterase 1
Target Function
Phosphodiesterase responsible for the U6 snRNA 3' end processing. Acts as an exoribonuclease (RNase) responsible for trimming the poly(U) tract of the last nucleotides in the pre-U6 snRNA molecule, leading to the formation of mature U6 snRNA 3' end-terminated with a 2',3'-cyclic phosphate.
Target Involvement
Poikiloderma with neutropenia (PN)
Target Subcellular Location
Nucleus.
Target Protein Families
2H phosphoesterase superfamily, USB1 family
Target Synonyms
PN; Mpn1; HVSL1; hMpn1; hUsb1; C16orf57; USB1
Target Background
This gene encodes a protein with several conserved domains, however, its exact function is not known. Mutations in this gene are associated with poikiloderma with neutropenia (PN), which shows phenotypic overlap with Rothmund-Thomson syndrome (RTS) caused by mutations in the RECQL4 gene. It is believed that this gene product interacts with RECQL4 protein via SMAD4 proteins, explaining the partial clinical overlap between PN and RTS. Alternatively spliced transcript variants encoding different isoforms have been noted for this gene.
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