LRPPRC Antibody (PACO18174)
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주문- SKU:
- PACO18174
- Product type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Application:
- ELISA
- Application:
- IHC
- Antibody type:
- Polyclonal
- Conjugation:
- Unconjugated
Frequently bought together:
Description
Antibody Name: | LRPPRC Antibody (PACO18174) |
Antibody SKU: | PACO18174 |
Size: | 50ul |
Host Species: | Rabbit |
Tested Applications: | ELISA, IHC |
Recommended Dilutions: | ELISA:1:1000-1:2000, IHC:1:15-1:50 |
Species Reactivity: | Human, Mouse, Rat |
Immunogen: | Synthetic peptide of human LRPPRC |
Form: | Liquid |
Storage Buffer: | -20°C, pH7.4 PBS, 0.05% NaN3, 40% Glycerol |
Purification Method: | Antigen affinity purification |
Clonality: | Polyclonal |
Isotype: | IgG |
Conjugate: | Non-conjugated |
![]() | The image on the left is immunohistochemistry of paraffin-embedded Human ovarian cancer tissue using PACO18174(LRPPRC Antibody) at dilution 1/15, on the right is treated with synthetic peptide. (Original magnification: x200). |
![]() | The image on the left is immunohistochemistry of paraffin-embedded Human colon cancer tissue using PACO18174(LRPPRC Antibody) at dilution 1/15, on the right is treated with synthetic peptide. (Original magnification: x200). |
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. |
Synonyms: | leucine-rich pentatricopeptide repeat containing |
UniProt Protein Function: | LRPPRC: May play a role in RNA metabolism in both nuclei and mitochondria. In the nucleus binds to HNRPA1-associated poly(A) mRNAs and is part of nmRNP complexes at late stages of mRNA maturation which are possibly associated with nuclear mRNA export. May bind mature mRNA in the nucleus outer membrane. In mitochondria binds to poly(A) mRNA. Plays a role in translation or stability of mitochondrially encoded cytochrome c oxidase (COX) subunits. May be involved in transcription regulation. Cooperates with PPARGC1A to regulate certain mitochondrially encoded genes and gluconeogenic genes and may regulate docking of PPARGC1A to transcription factors. Seems to be involved in the transcription regulation of the multidrug-related genes MDR1 and MVP. Part of a nuclear factor that binds to the invMED1 element of MDR1 and MVP gene promoters. Binds single-stranded DNA. Defects in LRPPRC are the cause of Leigh syndrome French- Canadian type (LSFC). 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 liver mitochondria and a specifically reduced translation of COX subunits MT-CO1/COXI and MT-CO3 (COXIII). |
UniProt Protein Details: | Protein type:RNA-binding Chromosomal Location of Human Ortholog: 2p21 Cellular Component: nucleoplasm; nuclear outer membrane; microtubule; condensed nuclear chromosome; cytoskeleton; mitochondrion; membrane; perinuclear region of cytoplasm; nuclear inner membrane; nucleus; ribonucleoprotein complex Molecular Function:actin filament binding; protein binding; RNA binding; microtubule binding; beta-tubulin binding; single-stranded DNA binding Biological Process: mRNA transport; transcription, DNA-dependent; regulation of transcription, DNA-dependent; mitochondrion transport along microtubule Disease: Leigh Syndrome, French Canadian Type |
NCBI Summary: | 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] |
UniProt Code: | P42704 |
NCBI GenInfo Identifier: | 156632706 |
NCBI Gene ID: | 10128 |
NCBI Accession: | P42704.3 |
UniProt Related Accession: | P42704 |
Molecular Weight: | ~ 130kDa |
NCBI Full Name: | Leucine-rich PPR motif-containing protein, mitochondrial |
NCBI Synonym Full Names: | leucine rich pentatricopeptide repeat containing |
NCBI Official Symbol: | LRPPRC |
NCBI Official Synonym Symbols: | LSFC; GP130; LRP130; CLONE-23970 |
NCBI Protein Information: | leucine-rich PPR motif-containing protein, mitochondrial |
UniProt Protein Name: | Leucine-rich PPR motif-containing protein, mitochondrial |
UniProt Synonym Protein Names: | 130 kDa leucine-rich protein; LRP 130; GP130 |
Protein Family: | Leucine-rich PPR motif-containing protein |
UniProt Gene Name: | LRPPRC |
UniProt Entry Name: | LPPRC_HUMAN |