MPU1 rabbit pAb

MPU1 rabbit pAb

AO-06-ES14716-100

MPU1 rabbit pAb 100μL

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Antibody Product Overview

ELK.NoES14716
Product nameMPU1 rabbit pAb
ReactivityHuman; Mouse
ApplicationsWB
Other name
Size100μL
Unit price ($)248
Human gene ID9526
Human Swiss-ProtO75352
SourceRabbit
IsotypeIgG
TargetMPU1
Fields
Gene nameMPDU1
Protein nameMPU1
Human gene linkView Human Gene
Human Swiss linkView Human Swiss-Prot
Mouse gene ID
Mouse gene link
Mouse Swiss-ProtQ9R0Q9
Mouse Swiss linkView Mouse Swiss-Prot
Rat gene ID
Rat gene link
Rat Swiss-Prot
Rat Swiss link
ImmunogenSynthesized peptide derived from human MPU1 AA range: 164-214
SpecificityThis antibody detects endogenous levels of MPU1 at Human/Mouse
FormulationLiquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide.
ClonalityPolyclonal
DilutionWB 1:500-2000
PurificationThe antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen.
Concentration1 mg/ml
Storage stability-20°C/1 year
Molecular Weight (Da)27kD
Observed band (KD)
BackgroundThis 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],
Functiondisease:Defects in MPDU1 are the cause of congenital disorder of glycosylation type 1F (CDG1F) [MIM:609180]. 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:Not known. May be involved in the synthesis of the sugar donor Dol-P-Man which is required in the synthesis of N-linked and O-linked oligosaccharides and for that of GPI anchors.,similarity:Belongs to the MPDU1 family.,similarity:Conta
Subcellular locationMembrane ; Multi-pass membrane protein .
Expression

Additional Images

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Western blot analysis of lysates from THP-1 cells, primary antibody was diluted at 1:1000, 4°over night
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: AO-06-ES14716-100
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