USB1 Polyklonaler Antikörper
USB1 Polyklonal Antikörper für WB, Indirect ELISA
Wirt / Isotyp
Kaninchen / IgG
Getestete Reaktivität
human
Anwendung
WB, Indirect ELISA
Konjugation
Unkonjugiert
Kat-Nr. : 21229-1-PBS
Synonyme
Geprüfte Anwendungen
Produktinformation
21229-1-PBS bindet in WB, Indirect ELISA USB1 und zeigt Reaktivität mit human
| Getestete Reaktivität | human |
| Wirt / Isotyp | Kaninchen / IgG |
| Klonalität | Polyklonal |
| Typ | Antikörper |
| Immunogen | USB1 fusion protein Ag15689 |
| Vollständiger Name | chromosome 16 open reading frame 57 |
| Berechnetes Molekulargewicht | 265 aa, 30 kDa |
| Beobachtetes Molekulargewicht | 31 kDa |
| GenBank-Zugangsnummer | BC004415 |
| Gene symbol | C16orf57 |
| Gene ID (NCBI) | 79650 |
| Konjugation | Unkonjugiert |
| Form | Liquid |
| Reinigungsmethode | Antigen-Affinitätsreinigung |
| Lagerungspuffer | PBS only |
| Lagerungsbedingungen | Store at -80°C. 20ul Größen enthalten 0,1% BSA. |
Hintergrundinformationen
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.

