GCS1 anticorps (N-Term)

N° du produit ABIN357669

Cet anticorps anti-GCS1 est un anticorps Lapin Polyclonal détectant GCS1 dans WB et EIA. Adapté pour Humain.

Aperçu rapide pour GCS1 anticorps (N-Term) (ABIN357669)

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

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp GCS1 est non-conjugé

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

Détail du produit anti-GCS1 anticorps

Épitope: N-Term

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

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

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

Isotype: Ig Fraction

Information d'application

Indications d'application: 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.

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 0.25 mg/mL

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

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.

Conseil sur la manipulation: Avoid repeated freezing and thawing.

Stock: 4 °C/-20 °C

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

Détails sur GCS1

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

Autre désignation: MOGS / 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.Synonyms: Mannosyl-oligosaccharide glucosidase, Processing A-glucosidase I

Poids moléculaire: 91840 Da

ID gène: 7841, 5874

UniProt: Q13724

Pathways: SARS-CoV-2 Protein Interactome