The ALG11 Polyclonal Antibody (PAC056470) is a valuable tool for researchers studying ALG11, an essential enzyme involved in protein glycosylation. This antibody, produced in rabbits, demonstrates high reactivity with human samples and is validated for use in applications such as Western blotting. By binding to the ALG11 protein, this antibody enables precise detection and analysis in a variety of cell types, making it ideal for investigations in molecular biology and metabolic disorders.ALG11 plays a crucial role in the process of N-linked glycosylation, which is essential for proper protein folding and function. Dysfunction of ALG11 has been linked to a variety of metabolic disorders and congenital diseases, making it an attractive target for therapeutic research.
By studying the activity of ALG11, researchers can gain insights into potential treatment options for individuals affected by these conditions.In conclusion, the ALG11 Polyclonal Antibody (PAC056470) is a valuable tool for researchers interested in exploring the role of ALG11 in protein glycosylation and related metabolic pathways. Its high reactivity and specificity make it a reliable choice for investigations into molecular mechanisms underlying various diseases and disorders.
IHC image of PACO56470 diluted at 1:250 and staining in paraffin-embedded human adrenal gland tissue performed on a Leica BondTM system. After dewaxing and hydration, antigen retrieval was mediated by high pressure in a citrate buffer (pH 6.0). Section was blocked with 10% normal goat serum 30min at RT. Then primary antibody (1% BSA) was incubated at 4°C overnight. The primary is detected by a biotinylated secondary antibody and visualized using an HRP conjugated SP system.
Immunofluorescence staining of HepG2 cells with PACO56470 at 1:105, counter-stained with DAPI. The cells were fixed in 4% formaldehyde, permeabilized using 0.2% Triton X-100 and blocked in 10% normal Goat Serum. The cells were then incubated with the antibody overnight at 4°C. The secondary antibody was Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L).
Background:
Mannosyltransferase involved in the last steps of the synthesis of Man5GlcNAc(2)-PP-dolichol core oligosaccharide on the cytoplasmic face of the endoplasmic reticulum. Catalyzes the addition of the 4th and 5th mannose residues to the dolichol-linked oligosaccharide chain.
ALG11: Mannosyltransferase involved in the last steps of the synthesis of Man5GlcNAc(2)-PP-dolichol core oligosaccharide on the cytoplasmic face of the endoplasmic reticulum. Catalyzes the addition of the 4th and 5th mannose residues to the dolichol- linked oligosaccharide chain. Defects in ALG11 are the cause of congenital disorder of glycosylation type 1P (CDG1P). A multisystem disorder caused by a defect in glycoprotein biosynthesis and characterized by under-glycosylated serum glycoproteins. Congenital disorders of glycosylation result in a wide variety of clinical features, such as defects in the nervous system development, psychomotor retardation, dysmorphic features, hypotonia, coagulation disorders, and immunodeficiency. The broad spectrum of features reflects the critical role of N-glycoproteins during embryonic development, differentiation, and maintenance of cell functions. Belongs to the glycosyltransferase group 1 family. Glycosyltransferase 4 subfamily.Protein type: EC 2.4.1.131; Transferase; Membrane protein, multi-pass; Glycan Metabolism - N-glycan biosynthesis; Membrane protein, integralChromosomal Location of Human Ortholog: 13q14.2Cellular Component: membraneDisease: Congenital Disorder Of Glycosylation, Type Ip
UniProt Protein Details:
NCBI Summary:
This gene encodes a GDP-Man:Man3GlcNAc2-PP-dolichol-alpha1,2-mannosyltransferase which is localized to the cytosolic side of the endoplasmic reticulum (ER) and catalyzes the transfer of the fourth and fifth mannose residue from GDP-mannose (GDP-Man) to Man3GlcNAc2-PP-dolichol and Man4GlcNAc2-PP-dolichol resulting in the production of Man5GlcNAc2-PP-dolichol. Mutations in this gene are associated with congenital disorder of glycosylation type Ip (CDGIP). This gene overlaps but is distinct from the UTP14, U3 small nucleolar ribonucleoprotein, homolog C (yeast) gene. A pseudogene of the GDP-Man:Man3GlcNAc2-PP-dolichol-alpha1,2-mannosyltransferase has been identified on chromosome 19. [provided by RefSeq, Aug 2010]
