GCS1 Antikörper (N-Term)

Produktnummer ABIN357669

Dieses Anti-GCS1-Antikörper ist ein Kaninchen Polyklonal-Antikörper zur Detektion von GCS1 in WB und EIA. Geeignet für Human.

Kurzübersicht für GCS1 Antikörper (N-Term) (ABIN357669)

Target: GCS1 (MOGS) (Mannosyl-Oligosaccharide Glucosidase (MOGS))

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GCS1 Antikörper ist unkonjugiert

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

Produktdetails anti-GCS1 Antikörper

Bindungsspezifität: N-Term

Spezifität: This antibody is specific to GCS1 (N-term).

Aufreinigung: Protein G Chromatography, eluted with high and low pH buffers and neutralized immediately, followed by dialysis against PBS.

Immunogen: This antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide selected from the N-terminal region of human GCS1.

Isotyp: Ig Fraction

Anwendungsinformationen

Applikationshinweise: ELISA: 1/1,000. Western Blot: 1/50-1/100.
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user.

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Konzentration: 0.25 mg/mL

Buffer: PBS with 0.09 % (W/V) Sodium Azide as preservative.

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 GCS1

Target: GCS1 (MOGS) (Mannosyl-Oligosaccharide Glucosidase (MOGS))

Andere Bezeichnung: MOGS / GCS1

Hintergrund: GCS1 cleaves the distal alpha 1,2-linked glucose residue from the Glc(3)Man(9)GlcNAc(2) oligosaccharide precursor in a highly specific manner. Defects in GCS1 are the cause of type IIb congenital disorder of glycosylation (CDGIIb). This syndrome is also known as glucosidase I deficiency and is characterized by marked generalized hypotonia and hypomotility of the neonate, dysmorphic features, including a prominent occiput, short palpebral fissures, retrognathia, high arched palate, generalized edema, and hypoplastic genitalia. Symptoms include hepatomegaly, hypoventilation, feeding problems and seizures. The clinical course is progressive and survival is at most a few months.Synonyms: Mannosyl-oligosaccharide glucosidase, Processing A-glucosidase I

Molekulargewicht: 91840 Da

Gen-ID: 7841, 5874

UniProt: Q13724

Pathways: SARS-CoV-2 Protein Interaktom