GMPPB antibody (AA 111-210) (AbBy Fluor® 750)

Catalog No. ABIN5003702

Product Details anti-GMPPB Antibody (AbBy Fluor® 750)

Target: GMPPB (GDP-Mannose Pyrophosphorylase B (GMPPB))

Binding Specificity: AA 111-210

Reactivity: Human

Host: Rabbit

Clonality: Polyclonal

Conjugate: This GMPPB antibody is conjugated to AbBy Fluor® 750

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Predicted Reactivity: Human,Mouse,Rat,Cow,Sheep,Pig,Horse,Rabbit

Purification: Purified by Protein A.

Immunogen: KLH conjugated synthetic peptide derived from human GMPPB

Isotype: IgG

Application Details

Application Notes: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Handling

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Preservative: ProClin

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

Storage: -20 °C

Storage Comment: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Expiry Date: 12 months

Target Details for GMPPB

Target: GMPPB (GDP-Mannose Pyrophosphorylase B (GMPPB))

Alternative Name: GMPPB (GMPPB Products)

Background:

Synonyms: GDP mannose pyrophosphorylase B, GDP-mannose pyrophosphorylase B, GMPPB, GMPPB_HUMAN, GTP-mannose-1-phosphate guanylyltransferase beta, KIAA1851, Mannose 1 phosphate guanylyltransferase, Mannose-1-phosphate guanyltransferase beta.

Background: GMPPB is a 360 amino acid protein that belongs to the transferase hexapeptide repeat family and is involved in protein modification pathways. Functioning as a GDP-mannose pyrophosphorylase, GMPPB enzymatically catalyzes the conversion of mannose-1-phosphate and GTP to GDP-mannose and a free phosphate, a reaction that is involved in the production of N-linked oligosaccharides. Defects in the gene encoding GMPPB that cause errors in the glycosylation pathway may lead to congenital disorders of glycosylation (CDG). CDGs are multisystemic diseases that often involve both the central and peripheral nervous systems and are often characterized by endocrine and coagulation disorders. GMPPB is expressed as two isoforms due to alternative splicing events.

Gene ID: 29925