Price range: $268.00 through $328.00
Background:
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],
| Size | 50μL/100μL |
| Target | ALG1 |
| Reactivity | Human, Mouse |
| Applications | WB |
| MW(Calculated) | 51kD |
| MW(Observed) | |
| Host Species | Rabbit |
| Isotype | IgG |
| Conjugate/Modification | Unmodified |
| Recommended Dilution Ratio | WB 1:500-2000 |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Purification | The antibody was affinity-purified from rabbit antiserum by affinity-chromatography using epitope-specific immunogen. |
| Storage | -15°C to -25°C/1 year(Do not lower than -25°C) |
| Concentration | 1 mg/ml |
| Clonality | Polyclonal |
| Clone Number | |
| Immunogen | Synthesized peptide derived from human ALG1 AA range: 180-230 |
| Specificity | This antibody detects endogenous levels of ALG1 at Human/Mouse |
| Gene Name | ALG1 HMAT1 HMT1 PSEC0061 UNQ861/PRO1870 |
| Protein Name | ALG1 |
| Other Name | |
| Species | Human |
| Gene ID | 56052 |
| Uniprot | Q9BT22, |
| Species.1 | Mouse |
| Gene ID.1 | 208211 |
| Uniprot.1 | Q921Q3 |
| Species.2 | |
| Gene ID.2 | |
| Uniprot.2 | |
| Cellular Localization | Endoplasmic reticulum membrane; Single-pass type II membrane protein . |
| Tissue Expression | |
| Signaling_pathway | |
| Research Areas | >>N-Glycan biosynthesis; / >>Various types of N-glycan biosynthesis; / >>Metabolic pathways |
| Function | Catalytic activity:GDP-mannose + chitobiosyldiphosphodolichol = GDP + beta-1,4-D-mannosylchitobiosyldiphosphodolichol.,Disease:Defects in ALG1 are the cause of congenital disorder of glycosylation type 1K (CDG1K) [MIM:608540]. 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.,Function: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.,online information:GlycoGene database,pathway:Protein modification; protein glycosylation.,similarity:Belongs to the glycosyltransferase 1 family., |
