C16orf57 Antikörper (Internal Region)

Produktnummer ABIN950776

Produktdetails anti-C16orf57 Antikörper

Target: C16orf57 (USB1) (U6 SnRNA Biogenesis 1 (USB1))

Bindungsspezifität: AA 154-167, Internal Region

Reaktivität: Human, Schwein

Wirt: Ziege

Klonalität: Polyklonal

Konjugat: Dieser C16orf57 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB), Enzyme Immunoassay (EIA)

Sequenz: C-TANQVKIYTN QEKT

Spezifität: This antibody is expected to recognize both reported isoforms (NP_078874.2, NP_001182231.1).

Kreuzreaktivität (Details): Species reactivity (expected):Pig.
Species reactivity (tested):Human, Mouse, Rat.

Aufreinigung: Ammonium Sulphate Precipitation followed by Antigen Affinity Chromatography using the immunizing peptide

Immunogen: Peptide with sequence from the internal region of the protein sequence according to NP_078874.2, NP_001182231.1.

Anwendungsinformationen

Applikationshinweise: Optimal working dilution should be determined by the investigator.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Konzentration: 0.5 mg/mL

Buffer: Tris saline, pH ~7.3 containing 0.02 % Sodium Azide as preservative and 0.5 % BSA as stabilizer

Konservierungsmittel: Sodium azide

Vorsichtsmaßnahmen: This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Handhabung: Avoid repeated freezing and thawing.

Lagerung: 4 °C/-20 °C

Informationen zur Lagerung: Store the antibody undiluted at 2-8 °C for one month or (in aliquots) at -20 °C for longer.

Details zu C16orf57

Target: C16orf57 (USB1) (U6 SnRNA Biogenesis 1 (USB1))

Andere Bezeichnung: C16orf57 (USB1 Produkte)

Synonyme: C16orf57 antikoerper, HVSL1 antikoerper, Mpn1 antikoerper, PN antikoerper, hUsb1 antikoerper, C18H16orf57 antikoerper, AA960436 antikoerper, RGD1305215 antikoerper, c16orf57 antikoerper, C2H16orf57 antikoerper, zgc:91896 antikoerper, U6 snRNA biogenesis phosphodiesterase 1 antikoerper, U6 snRNA biogenesis 1 antikoerper, U6 snRNA biogenesis phosphodiesterase 1 L homeolog antikoerper, USB1 antikoerper, Usb1 antikoerper, usb1.L antikoerper, usb1 antikoerper

Hintergrund: Defects in C16orf57 are the cause of poikiloderma with neutropenia (PN) [MIM:604173]. PN is a genodermatosis characterized by poikiloderma, pachyonychia and chronic neutropenia. The disorder starts as a papular erythematous rash on the limbs during the first year of life. It gradually spreads centripetally and, as the papular rash resolves, hypo- and hyperpigmentation result, with development of telangiectasias. Another skin manifestation is pachyonychia, but alopecia and leukoplakia are distinctively absent. One of the most important extracutaneous symptoms is an increased susceptibility to infections, mainly affecting the respiratory system, primarily due to a chronic neutropenia and to neutrophil functional defects. Bone marrow abnormalities account for neutropenia and may evolve into myelodysplasia associated with the risk of leukemic transformation. Poikiloderma with neutropenia shows phenotypic overlap with Rothmund-Thomson syndrome.Synonyms: FLJ13154, UPF0406 protein C16orf57

Gen-ID: 79650

NCBI Accession: NP_078874