The ALG6 Polyclonal Antibody (PAC019092) is a valuable tool for researchers studying ALG6, an enzyme involved in protein glycosylation processes within the endoplasmic reticulum. This antibody, raised in rabbits, exhibits high specificity and sensitivity for detecting ALG6 in human samples, making it suitable for use in various research applications such as Western blotting.ALG6 plays a crucial role in the biosynthesis of glycoproteins, a process essential for proper protein folding and function. Dysregulation of ALG6 has been implicated in various diseases, including congenital disorders of glycosylation and neurodegenerative conditions.
By targeting ALG6 with this antibody, researchers can gain insights into its function and explore its potential as a therapeutic target for treating these disorders.The ALG6 Polyclonal Antibody is a reliable tool for investigating the role of ALG6 in cellular processes and disease pathogenesis. Its ability to specifically detect ALG6 in human samples makes it an invaluable resource for researchers working in the fields of biochemistry, cell biology, and genetics.
Antibody Name:
ALG6 Antibody (PACO19092)
Antibody SKU:
PACO19092
Size:
50ul
Host Species:
Rabbit
Tested Applications:
ELISA, WB
Recommended Dilutions:
ELISA:1:1000-1:2000, WB:1:200-1:1000
Species Reactivity:
Human, Mouse, Rat
Immunogen:
Synthetic peptide of human ALG6
Form:
Liquid
Storage Buffer:
-20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol
Purification Method:
Antigen affinity purification
Clonality:
Polyclonal
Isotype:
IgG
Conjugate:
Non-conjugated
Gel: 10%SDS-PAGE, Lysate: 40 μg, Lane: Human lymphoma tissue, Primary antibody: PACO19092(ALG6 Antibody) at dilution 1/350, Secondary antibody: Goat anti rabbit IgG at 1/8000 dilution, Exposure time: 30 seconds.
Background:
This gene encodes a member of the ALG6/ALG8 glucosyltransferase family. The encoded protein catalyzes the addition of the first glucose residue to the growing lipid-linked oligosaccharide precursor of N-linked glycosylation. Mutations in this gene are associated with congenital disorders of glycosylation type Ic.
Synonyms:
ALG6, alpha-1,3-glucosyltransferase
UniProt Protein Function:
ALG6: Adds the first glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation. Transfers glucose from dolichyl phosphate glucose (Dol-P-Glc) onto the lipid-linked oligosaccharide Man(9)GlcNAc(2)-PP-Dol. Defects in ALG6 are the cause of congenital disorder of glycosylation type 1C (CDG1C); also known as carbohydrate-deficient glycoprotein syndrome type V. CDGs are metabolic deficiencies in glycoprotein biosynthesis that usually cause severe mental and psychomotor retardation. They are characterized by under-glycosylated serum glycoproteins. CDG1C patients have muscular hypotonia, show a delayed statomotor development and are mentally retarded. CDG1C is biochemically characterized by an accumulation of dolichyl pyrophosphate-linked Man(9)GlcNAc(2) in the endoplasmic reticulum. Belongs to the ALG6/ALG8 glucosyltransferase family.Protein type: Membrane protein, integral; Glycan Metabolism - N-glycan biosynthesis; EC 2.4.1.267; Membrane protein, multi-pass; TransferaseChromosomal Location of Human Ortholog: 1p31.3Cellular Component: endoplasmic reticulum membrane; membraneMolecular Function: dolichyl pyrophosphate Man9GlcNAc2 alpha-1,3-glucosyltransferase activity; dolichyl-phosphate-glucose-glycolipid alpha-glucosyltransferase activity; glucosyltransferase activityBiological Process: dolichol-linked oligosaccharide biosynthetic process; oligosaccharide-lipid intermediate assembly; protein amino acid N-linked glycosylationDisease: Congenital Disorder Of Glycosylation, Type Ic
UniProt Protein Details:
NCBI Summary:
This gene encodes a member of the ALG6/ALG8 glucosyltransferase family. The encoded protein catalyzes the addition of the first glucose residue to the growing lipid-linked oligosaccharide precursor of N-linked glycosylation. Mutations in this gene are associated with congenital disorders of glycosylation type Ic. [provided by RefSeq, Jul 2008]