GCS1 antibody (N-Term)

Catalog No. ABIN389016

Product Details anti-GCS1 Antibody

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

Binding Specificity: AA 1-30, N-Term

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GCS1 antibody is un-conjugated

Application: Western Blotting (WB)

Purification: 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.

Clone: RB4961-4962

Isotype: Ig Fraction

Application Details

Application Notes: WB: 1:1000

Restrictions: For Research Use only

Handling

Format: Liquid

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

Preservative: Sodium azide

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

Storage: 4 °C,-20 °C

Storage Comment: 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.

Expiry Date: 6 months

Target Details for GCS1

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

Alternative Name: 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.

Molecular Weight: 91918

Gene ID: 7841

NCBI Accession: NP_001139630, NP_006293

UniProt: Q13724

Pathways: SARS-CoV-2 Protein Interactome