Rabbit anti-Human ALG8 Polyclonal Antibody | anti-ALG8 antibody

ALG8, NT (ALG8, Probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase, Asparagine-linked glycosylation protein 8 homolog, Dol-P-Glc:Glc(1)Man(9)GlcNAc(2)-PP-dolichyl alpha-1,3-glucosyltransferase, Dolichyl-P-Glc:Glc1Man9GlcNAc2-PP-

Cat. Num. AAA6272938

• Gene Names: ALG8; CDG1H; PCLD3
• Reactivity: Human
• Applications: Western Blot, ELISA
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: ALG8; Polyclonal Antibody; NT (ALG8; Probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1; 3-glucosyltransferase; Asparagine-linked glycosylation protein 8 homolog; Dol-P-Glc:Glc(1)Man(9)GlcNAc(2)-PP-dolichyl alpha-1; Dolichyl-P-Glc:Glc1Man9GlcNAc2-PP-; Dolichyl-P-Glc:Glc1Man9GlcNAc2-PP-dolichyl glucosyltransferase; anti-ALG8 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Alkaline Phosphatase (AP).
• Sequence Length: 526

• Applicable Applications for anti-ALG8 antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:100-500; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: ALG8 antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 14-41 amino acids from the N-terminal region of human ALG8.
• Conjugate: AP
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(ALG8 Antibody (N-term) western blot analysis in MDA-MB453 cell line lysates (35ug/lane).This demonstrates the ALG8 antibody detected the ALG8 protein (arrow).)

Related Product Information for anti-ALG8 antibody

This gene encodes a member of the ALG6/ALG8 glucosyltransferase family. The encoded protein catalyzes the addition of the second glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation of proteins. Mutations in this gene have been associated with congenital disorder of glycosylation type Ih (CDG-Ih). Alternatively spliced transcript variants encoding different isoforms have been identified.

Product Categories/Family for anti-ALG8 antibody

Antibodies; Enzymes

References

Rose, J.E., et al. Mol. Med. 16 (7-8), 247-253 (2010): Stolting, T., et al. Mol. Genet. Metab. 98(3):305-309(2009). Jaeken, J., et al. Curr. Opin. Pediatr. 16(4):434-439(2004). Schollen, E., et al. J. Med. Genet. 41(7):550-556(2004). Jaeken, J. J. Inherit. Metab. Dis. 27(3):423-426(2004).

NCBI and Uniprot Product Information

• NCBI GI #: 56121818
• NCBI GeneID: 79053
• NCBI Accession #: NP_076984.2
• NCBI GenBank Nucleotide #: NP_076984.2
• UniProt Accession #: Q9BVK2
• NCBI Official Full Name: probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase isoform a
• NCBI Official Synonym Full Names: ALG8 alpha-1,3-glucosyltransferase
• NCBI Official Symbol: ALG8
• NCBI Official Synonym Symbols: CDG1H; PCLD3
• NCBI Protein Information: probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase
• UniProt Protein Name: Probable dolichyl pyrophosphate Glc1Man9GlcNAc2 alpha-1,3-glucosyltransferase
• Protein Family: Glycosyltransferase
• UniProt Gene Name: ALG8

NCBI Description

This gene encodes a member of the ALG6/ALG8 glucosyltransferase family. The encoded protein catalyzes the addition of the second glucose residue to the lipid-linked oligosaccharide precursor for N-linked glycosylation of proteins. Mutations in this gene have been associated with congenital disorder of glycosylation type Ih (CDG-Ih). Alternatively spliced transcript variants encoding different isoforms have been identified. [provided by RefSeq, Jul 2008]

Uniprot Description

ALG8: Adds the second 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 Glc(1)Man(9)GlcNAc(2)-PP-Dol. Defects in ALG8 are the cause of congenital disorder of glycosylation type 1H (CDG1H). CDGs are a family of severe inherited diseases caused by a defect in protein N- glycosylation. They are characterized by under-glycosylated serum proteins. These multisystem disorders present with a wide variety of clinical features, such as disorders of 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 ALG6/ALG8 glucosyltransferase family.

Protein type: EC 2.4.1.265; Glycan Metabolism - N-glycan biosynthesis; Membrane protein, integral; Membrane protein, multi-pass; Transferase

Chromosomal Location of Human Ortholog: 11q14.1

Cellular Component: endoplasmic reticulum membrane; integral component of membrane

Molecular Function: alpha-1,3-mannosyltransferase activity; dolichyl pyrophosphate Man9GlcNAc2 alpha-1,3-glucosyltransferase activity; dolichyl-phosphate-glucose-glycolipid alpha-glucosyltransferase activity; protein binding

Biological Process: dolichol-linked oligosaccharide biosynthetic process; oligosaccharide-lipid intermediate biosynthetic process; protein N-linked glycosylation

Disease: Congenital Disorder Of Glycosylation, Type Ih