GCS1 anticorps (C-Term)

N° du produit ABIN389017

Cet anticorps Lapin Polyclonal détecte spécifiquement GCS1 dans WB. Il présente une réactivité envers Humain et Souris.

Aperçu rapide pour GCS1 anticorps (C-Term) (ABIN389017)

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

Reactivité: Humain, Souris

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GCS1 est non-conjugé

Application: Western Blotting (WB)

Clone: RB4963-4964

Détail du produit anti-GCS1 anticorps

Épitope: AA 796-826, C-Term

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

Immunogène: This GCS1 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 796-826 amino acids from the C-terminal region of human GCS1.

Isotype: Ig Fraction

Information d'application

Indications d'application: WB: 1:1000

Restrictions: For Research Use only

Stockage

Format: Liquid

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

Agent conservateur: Sodium azide

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

Stock: 4 °C,-20 °C

Stockage commentaire: 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.

Date de péremption: 6 months

Détails sur GCS1

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

Autre désignation: GCS1

Sujet: 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.

Poids moléculaire: 91918

ID gène: 7841

NCBI Accession: NP_001139630, NP_006293

UniProt: Q13724

Pathways: SARS-CoV-2 Protein Interactome