Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded human brain tissue using MBS7005700 at dilution of 1:100)
Rabbit anti-Human MPDU1 Polyclonal Antibody | anti-MPDU1 antibody
MPDU1 Antibody
Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded human brain tissue using MBS7005700 at dilution of 1:100)
Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded human brain tissue using MBS7005700 at dilution of 1:100)
Immunofluorescence (IF)
(Immunofluorescent analysis of U251 cells using MBS7005700 at a dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L))
Immunofluorescence (IF)
(Immunofluorescent analysis of U251 cells using MBS7005700 at a dilution of 1:100 and Alexa Fluor 488-congugated AffiniPure Goat Anti-Rabbit IgG(H+L))
NCBI and Uniprot Product Information
NCBI Description
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 Description
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, integral
Chromosomal Location of Human Ortholog: 17p13.1-p12
Cellular Component: endoplasmic reticulum; endoplasmic reticulum membrane; integral to membrane; membrane; mitochondrion
Molecular Function: protein binding
Biological Process: dolichol-linked oligosaccharide biosynthetic process; oligosaccharide biosynthetic process; protein folding; transport
Disease: Congenital Disorder Of Glycosylation, Type If
Research Articles on MPDU1
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Product Notes
The MPDU1 mpdu1 (Catalog #AAA7005700) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The MPDU1 Antibody reacts with Human and may cross-react with other species as described in the data sheet. AAA Biotech's MPDU1 can be used in a range of immunoassay formats including, but not limited to, ELISA (EIA), Immunohistochemistry (IHC), Immunofluorescence (IF). Researchers should empirically determine the suitability of the MPDU1 mpdu1 for an application not listed in the data sheet. Researchers commonly develop new applications and it is an integral, important part of the investigative research process. It is sometimes possible for the material contained within the vial of "MPDU1, Polyclonal Antibody" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
All products in the AAA Biotech catalog are strictly for research-use only, and are absolutely not suitable for use in any sort of medical, therapeutic, prophylactic, in-vivo, or diagnostic capacity. By purchasing a product from AAA Biotech, you are explicitly certifying that said products will be properly tested and used in line with industry standard. AAA Biotech and its authorized distribution partners reserve the right to refuse to fulfill any order if we have any indication that a purchaser may be intending to use a product outside of our accepted criteria.Disclaimer
Though we do strive to guarantee the information represented in this datasheet, AAA Biotech cannot be held responsible for any oversights or imprecisions. AAA Biotech reserves the right to adjust any aspect of this datasheet at any time and without notice. It is the responsibility of the customer to inform AAA Biotech of any product performance issues observed or experienced within 30 days of receipt of said product. To see additional details on this or any of our other policies, please see our Terms & Conditions page.Item has been added to Shopping Cart
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