GCS1 antibody (C-Term)

Catalog No. ABIN357670

Product Details anti-GCS1 Antibody

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

Binding Specificity: C-Term

Reactivity: Human, Mouse

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GCS1 antibody is un-conjugated

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

Specificity: This antibody is specific to GCS1 (C-term).

Purification: 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 form the C-terminal region of human GCS1.

Isotype: Ig Fraction

Application Details

Application Notes: ELISA: 1/1,000. Western Blot: 1/100-1/500.
Other applications not tested.
Optimal dilutions are dependent on conditions and should be determined by the user.

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 0.25 mg/mL

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

Preservative: Sodium azide

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

Handling Advice: Avoid repeated freezing and thawing.

Storage: 4 °C/-20 °C

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

Target Details for GCS1

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

Alternative Name: MOGS / GCS1 (MOGS Products)

Background: 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

Molecular Weight: 91840 Da

Gene ID: 7841, 5874

UniProt: Q13724

Pathways: SARS-CoV-2 Protein Interactome