For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Affinity Purification

Store at -20 degree C. Avoid freeze / thaw cycles.
Buffer: PBS with 0.02% sodium azide, 50% glycerol, pH7.3.

Small volumes of anti-ALG1 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

The enzyme encoded by this gene catalyzes the first mannosylation step in the biosynthesis of lipid-linked oligosaccharides. This gene is mutated in congenital disorder of glycosylation type Ik.

Western Blot (WB), Immunohistochemistry (IHC), Immunofluoresence (IF)

WB 1:500 - 1:2000
IHC 1:50 - 1:200
IF 1:50 - 1:100

Western blot analysis of extracts of various cell lines, using ALG1 antibody at 1:1000 dilution.
Secondary antibody: HRP Goat Anti-Rabbit IgG (H+L) (MBS128200) at 1:10000 dilution.
Lysates/proteins: 25ug per lane.
Blocking buffer: 3% nonfat dry milk in TBST.
Detection: ECL Basic Kit.
Exposure time: 30s.

Immunofluorescence analysis of A549 cells using ALG1 antibody. Blue: DAPI for nuclear staining.

53kDa

ALG1, chitobiosyldiphosphodolichol beta-mannosyltransferase

chitobiosyldiphosphodolichol beta-mannosyltransferase

Chitobiosyldiphosphodolichol beta-mannosyltransferase

HMAT1; HMT1; MT-1; hMat-1??[Similar Products]

ALG1_HUMAN

The enzyme encoded by this gene catalyzes the first mannosylation step in the biosynthesis of lipid-linked oligosaccharides. This gene is mutated in congenital disorder of glycosylation type Ik. [provided by RefSeq, Dec 2008]

ALG1: Participates in the formation of the lipid-linked precursor oligosaccharide for N-glycosylation. Involved in assembling the dolichol-pyrophosphate-GlcNAc(2)-Man(5) intermediate on the cytoplasmic surface of the ER. Defects in ALG1 are the cause of congenital disorder of glycosylation type 1K (CDG1K). 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 glycosyltransferase group 1 family. Glycosyltransferase 33 subfamily.

Protein type: Transferase; Endoplasmic reticulum; EC 2.4.1.142; Membrane protein, integral; Glycan Metabolism - N-glycan biosynthesis

Chromosomal Location of Human Ortholog: 16p13.3

Cellular Component: endoplasmic reticulum membrane; membrane; endoplasmic reticulum; integral to membrane

Molecular Function: mannosyltransferase activity; chitobiosyldiphosphodolichol beta-mannosyltransferase activity

Biological Process: cellular protein metabolic process; dolichol-linked oligosaccharide biosynthetic process; lipopolysaccharide biosynthetic process; protein amino acid glycosylation; protein amino acid N-linked glycosylation via asparagine; post-translational protein modification

Disease: Congenital Disorder Of Glycosylation, Type Ik

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