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Antibody Product Overview
| ELK.No | ES14715 |
| Product name | MPV17 rabbit pAb |
| Reactivity | Human; Mouse;Rat |
| Applications | WB |
| Other name | |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 4358 |
| Human Swiss-Prot | P39210 |
| Source | Rabbit |
| Isotype | IgG |
| Target | MPV17 |
| Fields | >>Peroxisome |
| Gene name | MPV17 |
| Protein name | MPV17 |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 17527 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | P19258 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | 360463 |
| Rat gene link | View Rat Gene |
| Rat Swiss-Prot | Q5BK62 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | Synthesized peptide derived from human MPV17 AA range: 45-95 |
| Specificity | This antibody detects endogenous levels of MPV17 at Human/Mouse/Rat |
| Formulation | Liquid in PBS containing 50% glycerol, 0.5% BSA and 0.02% sodium azide. |
| Clonality | Polyclonal |
| Dilution | WB 1：500-2000 |
| Purification | The antibody was affinity-purified from rabbit serum by affinity-chromatography using specific immunogen. |
| Concentration | 1 mg/ml |
| Storage stability | -20°C/1 year |
| Molecular Weight (Da) | 19kD |
| Observed band (KD) | |
| Background | This gene encodes a mitochondrial inner membrane protein that is implicated in the metabolism of reactive oxygen species. Mutations in this gene have been associated with the hepatocerebral form of mitochondrial DNA depletion syndrome (MDDS). [provided by RefSeq, Jul 2008], |
| Function | disease:Defects in MPV17 are a cause of hepatocerebral mitochondrial DNA depletion syndrome (MDS) [MIM:251880]. MDS is a clinically heterogeneous group of disorders characterized by a reduction in mitochondrial DNA (mtDNA) copy number. Primary mtDNA depletion is inherited as an autosomal recessive trait and may affect single organs, typically muscle or liver, or multiple tissues. Individuals with the hepatocerebral form of mitochondrial DNA depletion syndrome have early progressive liver failure and neurologic abnormalities, hypoglycemia, and increased lactate in body fluids.,disease:Defects in MPV17 are the cause of Navajo neurohepatopathy (NN) [MIM:256810]. NN is an autosomal recessive disease that is prevalent among Navajo children in the southwestern United States. The major clinical features are hepatopathy, peripheral neuropathy, corneal anesthesia and scarring, acral mutilation, c |
| Subcellular location | Mitochondrion inner membrane ; Multi-pass membrane protein . |
| Expression | Ubiquitous. Expressed in pancreas, kidney, muscle, liver, lung, placenta, brain and heart. |
Additional Images
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Western blot analysis of lysates from HpeG2 cells, primary antibody was diluted at 1:1000, 4°over night
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: AO-06-ES14715-100
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