Anticorps Polyclonal de lapin anti-USB1

USB1 Polyclonal Antibody for WB, Indirect ELISA

Hôte / Isotype

Lapin / IgG

Réactivité testée

Humain

Applications

WB, Indirect ELISA

Conjugaison

Non conjugué

N° de cat : 21229-1-PBS

Synonymes

C16orf57, 3'-5' RNA exonuclease USB1, EC:4.6.1.-, hMpn1, hUsb1

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Galerie de données de validation

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  WB analysis using 21229-1-AP (same clone as 21229-1-PBS)

  Various lysates were subjected to SDS PAGE followed by western blot with 21229-1-AP (USB1 antibody) at dilution of 1:2000 incubated at room temperature for 1.5 hours. This data was developed using the same antibody clone with 21229-1-PBS in a different storage buffer formulation.

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Informations sur le produit

21229-1-PBS cible USB1 dans les applications de WB, Indirect ELISA et montre une réactivité avec des échantillons Humain

• Réactivité: Humain
• Hôte / Isotype: Lapin / IgG
• Clonalité: Polyclonal
• Type: Anticorps
• Immunogène: USB1 Protéine recombinante Ag15689
• Nom complet: chromosome 16 open reading frame 57
• Masse moléculaire calculée: 265 aa, 30 kDa
• Poids moléculaire observé: 31 kDa
• Numéro d’acquisition GenBank: BC004415
• Symbole du gène: C16orf57
• Identification du gène (NCBI): 79650
• Conjugaison: Non conjugué
• Forme: Liquide
• Méthode de purification: Purification par affinité contre l'antigène
• Tampon de stockage: PBS only
• Conditions de stockage: Store at -80°C. 20ul contiennent 0,1% de BSA.

Informations générales

USB1 (U6 snRNA biogenesis phosphodiesterase 1) is a key nuclease primarily located within the nucleus and responsible for RNA processing. Its most well-defined function is to catalyze the hydrolysis of the 2',3'-cyclic phosphate at the 3' end of the U6 small nuclear RNA (snRNA), generating an essential 3'-hydroxyl group. This process is crucial for spliceosome assembly and pre-mRNA splicing. Mutations in the USB1 gene directly cause cartilaginous-hair hypoplasia, a rare autosomal recessive genetic disorder. Affected individuals present with short-limbed dwarfism, sparse hair, immunodeficiency, and bone marrow failure, revealing the critical roles of USB1 in skeletal development, cell cycle regulation, and immune system function. Therefore, USB1 is not only a core factor in RNA metabolism but also an important disease-associated protein.

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