MPDU1 Antibody (PACO28306)
- SKU:
- PACO28306
- Product Type:
- Antibody
- Reactivity:
- Human
- Host Species:
- Rabbit
- Isotype:
- IgG
- Applications:
- ELISA
- Applications:
- IHC
- Applications:
- IF
- Antibody Type:
- Polyclonal Antibody
- Conjugation:
- Unconjugated
Description
| Antibody Name: | MPDU1 Antibody (PACO28306) |
| Antibody SKU: | PACO28306 |
| Size: | 50ug |
| Host Species: | Rabbit |
| Tested Applications: | ELISA, IHC, IF |
| Recommended Dilutions: | ELISA:1:2000-1:10000, IHC:1:20-1:200, IF:1:50-1:200 |
| Species Reactivity: | Human |
| Immunogen: | Recombinant Human Mannose-P-dolichol utilization defect 1 protein (1-36AA) |
| Form: | Liquid |
| Storage Buffer: | Preservative: 0.03% Proclin 300 Constituents: 50% Glycerol, 0.01M PBS, PH 7.4 |
| Purification Method: | >95%, Protein G purified |
| Clonality: | Polyclonal |
| Isotype: | IgG |
| Conjugate: | Non-conjugated |
 | Immunohistochemistry of paraffin-embedded human brain tissue using PACO28306 at dilution of 1:100. |
 | Immunofluorescent analysis of U251 cells using PACO28306 at dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L). |
| Background: | Required for normal utilization of mannose-dolichol phosphate (Dol-P-Man) in the synthesis of N-linked and O-linked oligosaccharides and GPI anchors. |
| Synonyms: | Mannose-P-dolichol utilization defect 1 protein (Suppressor of Lec15 and Lec35 glycosylation mutation homolog) (SL15), MPDU1 |
| UniProt Protein Function: | MPDU1: Required for normal utilization of mannose-dolichol phosphate (Dol-P-Man) in the synthesis of N-linked and O-linked oligosaccharides and GPI anchors. Defects in MPDU1 are the cause of congenital disorder of glycosylation type 1F (CDG1F). 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 MPDU1 (TC 2.A.43.3) family.Protein type: Membrane protein, multi-pass; Membrane protein, integralChromosomal Location of Human Ortholog: 17p13.1-p12Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane; integral to membrane; membrane; mitochondrionMolecular Function: protein bindingBiological Process: dolichol-linked oligosaccharide biosynthetic process; oligosaccharide biosynthetic process; protein folding; transportDisease: Congenital Disorder Of Glycosylation, Type If |
| UniProt Protein Details: | |
| NCBI Summary: | This gene encodes an endoplasmic reticulum membrane protein that is required for utilization of the mannose donor mannose-P-dolichol in the synthesis of lipid-linked oligosaccharides and glycosylphosphatidylinositols. Mutations in this gene result in congenital disorder of glycosylation type If. Alternative splicing results in multiple transcript variants. [provided by RefSeq, Dec 2008] |
| UniProt Code: | O75352 |
| NCBI GenInfo Identifier: | 116517313 |
| NCBI Gene ID: | 9526 |
| NCBI Accession: | NP_004861.2 |
| UniProt Secondary Accession: | O75352,Q9BUU8, B3KQP1, B4DT74 |
| UniProt Related Accession: | O75352 |
| Molecular Weight: | 19,752 Da |
| NCBI Full Name: | mannose-P-dolichol utilization defect 1 protein |
| NCBI Synonym Full Names: | mannose-P-dolichol utilization defect 1 |
| NCBI Official Symbol: | MPDU1 |
| NCBI Official Synonym Symbols: | SL15; CDGIF; Lec35; My008; PQLC5; PP3958; HBEBP2BPA |
| NCBI Protein Information: | mannose-P-dolichol utilization defect 1 protein |
| UniProt Protein Name: | Mannose-P-dolichol utilization defect 1 protein |
| UniProt Synonym Protein Names: | Suppressor of Lec15 and Lec35 glycosylation mutation homolog; SL15 |
| Protein Family: | Mannose-P-dolichol utilization defect 1 protein |
| UniProt Gene Name: | MPDU1 |
| UniProt Entry Name: | MPU1_HUMAN |
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