GCS1 Antikörper (N-Term)

Produktnummer ABIN389016

Produktdetails anti-GCS1 Antikörper

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

Bindungsspezifität: AA 1-30, N-Term

Reaktivität: Human

Wirt: Kaninchen

Klonalität: Polyklonal

Konjugat: Dieser GCS1 Antikörper ist unkonjugiert

Applikation: Western Blotting (WB)

Aufreinigung: This antibody is prepared by Saturated Ammonium Sulfate (SAS) precipitation followed by dialysis against PBS.

Immunogen: This GCS1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 1-30 amino acids from the N-terminal region of human GCS1.

Klon: RB4961-4962

Isotyp: Ig Fraction

Anwendungsinformationen

Applikationshinweise: WB: 1:1000

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Format: Liquid

Buffer: Purified polyclonal antibody supplied in PBS with 0.09 % (W/V) sodium azide.

Konservierungsmittel: Sodium azide

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

Lagerung: 4 °C,-20 °C

Informationen zur Lagerung: Maintain refrigerated at 2-8 °C for up to 6 months. For long term storage store at -20 °C in small aliquots to prevent freeze-thaw cycles.

Haltbarkeit: 6 months

Details zu GCS1

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

Andere Bezeichnung: GCS1 (MOGS Produkte)

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.

Molekulargewicht: 91918

Gen-ID: 7841

NCBI Accession: NP_001139630, NP_006293

UniProt: Q13724

Pathways: SARS-CoV-2 Protein Interaktom