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Rabbit anti-Human USB1 Polyclonal Antibody

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.

ADC-04952A

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

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Specifications


Cat.No ADC-04952A ClonalityPolyclonal
Host SpeciesRabbitTarget NameUSB1
Target SynonymsC16orf57 antibody; Chromosome 16 open reading frame 57 antibody; CP057_HUMAN antibody; FLJ13154 antibody; UPF0406 protein C16orf57 antibodyFormLiquid
Species ReactivityHumanIsotypeIgG
Storage Buffer0.01M PBS, 0.03% Proclin 300; Constituents: 50% Glycerol, PH 7.4Purification Method>95%, Protein G purified
ConjugateNon-conjugatedApplicationELISA, IHC
StorageUpon receipt

Immunogen Information


Immunogen DescriptionRecombinant Human U6 snRNA phosphodiesterase protein (1-90AA)Target SpeciesHuman
Immunogen SequenceComplete sequences for the immunogen, target protein, and peptides are available upon request.Uniprot IDQ9BQ65
Background Information
  • 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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