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Antibody Product Overview
| ELK.No | ES15126 |
| Product name | LPPRC rabbit pAb |
| Reactivity | Human; Mouse;Rat |
| Applications | WB;ELISA;IHC |
| Other name | |
| Size | 100μL |
| Unit price ($) | 248 |
| Human gene ID | 10128 |
| Human Swiss-Prot | P42704 |
| Source | Rabbit |
| Isotype | IgG |
| Target | LPPRC |
| Fields | |
| Gene name | LRPPRC LRP130 |
| Protein name | LPPRC |
| Human gene link | View Human Gene |
| Human Swiss link | View Human Swiss-Prot |
| Mouse gene ID | 72416 |
| Mouse gene link | View Mouse Gene |
| Mouse Swiss-Prot | Q6PB66 |
| Mouse Swiss link | View Mouse Swiss-Prot |
| Rat gene ID | 313867 |
| Rat gene link | View Rat Gene |
| Rat Swiss-Prot | Q5SGE0 |
| Rat Swiss link | View Rat Swiss-Prot |
| Immunogen | Synthesized peptide derived from human LPPRC AA range: 1329-1379 |
| Specificity | This antibody detects endogenous levels of LPPRC 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;IHC-p 1:50-300; ELISA 2000-20000 |
| 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) | 153kD |
| Observed band (KD) | |
| Background | This gene encodes a leucine-rich protein that has multiple pentatricopeptide repeats (PPR). The precise role of this protein is unknown but studies suggest it may play a role in cytoskeletal organization, vesicular transport, or in transcriptional regulation of both nuclear and mitochondrial genes. The protein localizes primarily to mitochondria and is predicted to have an N-terminal mitochondrial targeting sequence. Mutations in this gene are associated with the French-Canadian type of Leigh syndrome. [provided by RefSeq, Mar 2012], |
| Function | disease:Defects in LRPPRC are the cause of Leigh syndrome French-Canadian type (LSFC) [MIM:220111]. Leigh syndrome is a severe neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions that is commonly associated with systemic cytochrome c oxidase (COX) deficiency. In the Saguenay-Lac Saint Jean region of Quebec province in Canada, a biochemically distinct form of Leigh syndrome with COX deficiency has been described. Patients have been observed to have a developmental delay, hypotonia, mild facial dysmorphism, chronic well-compensated metabolic acidosis, and high mortality due to episodes of severe acidosis and coma. Enzyme activity was close to normal in kidney and heart, 50% of normal in fibroblasts and skeletal muscle, and nearly absent in brain and liver. LSFC patients show reduced (<30%) levels of LRPPRC in both fibroblast and live |
| Subcellular location | Mitochondrion. Nucleus, nucleoplasm. Nucleus inner membrane. Nucleus outer membrane. Seems to be predominantly mitochondrial. |
| Expression | Expressed ubiquitously. Expression is highest in heart, skeletal muscle, kidney and liver, intermediate in brain, non-mucosal colon, spleen and placenta, and lowest in small intestine, thymus, lung and peripheral blood leukocytes. |
Additional Images
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Western blot analysis of lysates from Hela cells, primary antibody was diluted at 1:1000, 4°over night
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Immunohistochemical analysis of paraffin-embedded human liver cancer. 1, Antibody was diluted at 1:200(4° overnight). 2, Tris-EDTA,pH9.0 was used for antigen retrieval. 3,Secondary antibody was diluted at 1:200(room temperature, 45min).
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: AO-06-ES15126-100
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