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The antibody against USB1 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-265 of human USB1 (NP_078874.2) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
The antibody against USB1 was raised in Rabbit using the recombinant fusion protein containing a sequence corresponding to amino acids 1-265 of human USB1 (NP_078874.2) as the immunogen. The polyclonal antibody exists as a isotype IgG, by affinity purification. This antibody has been validated on WB, ELISA.
| Cat.No | ADA-03535A | Clonality | Polyclonal |
|---|---|---|---|
| Host Species | Rabbit | Target Name | USB1 |
| Target Synonyms | PN; Mpn1; HVSL1; hMpn1; hUsb1; C16orf57; USB1 | Form | Liquid |
| Species Reactivity | Human, Mouse | Isotype | IgG |
| Storage Buffer | 50% Glycerol, PBS with 0.01% thimerosal, pH7.3. | Purification Method | Affinity purification |
| Positive Samples | HeLa, 293T, A-431, Mouse liver | Application | ELISA, WB |
| Immunogen Description | Recombinant fusion protein containing a sequence corresponding to amino acids 1-265 of human USB1 (NP_078874.2). | Target Species | Human |
|---|---|---|---|
| Uniprot ID | Q9BQ65 | Immunogen Sequence |
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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