Western Blot (WB)
(Host: RabbitTarget Name: LPPRCSample Type: 293T Whole Cell lysatesAntibody Dilution: 1.0ug/ml)
Rabbit LRPPRC Polyclonal Antibody | anti-LRPPRC antibody
LRPPRC Antibody - N-terminal region
Western Blot (WB)
(Host: RabbitTarget Name: LPPRCSample Type: 293T Whole Cell lysatesAntibody Dilution: 1.0ug/ml)
Western Blot (WB)
(Host: RabbitTarget Name: LPPRCSample Type: 293T Whole Cell lysatesAntibody Dilution: 1.0ug/ml)
Target Description: 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.
NCBI and Uniprot Product Information
NCBI Description
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 Description
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).
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
Research Articles on LRPPRC
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Product Notes
The LRPPRC lrpprc (Catalog #AAA3205642) is an Antibody produced from Rabbit and is intended for research purposes only. The product is available for immediate purchase. The LRPPRC Antibody - N-terminal region reacts with Cow, Dog, Guinea Pig, Horse, Human, Mouse, Rabbit, Rat, Zebrafish and may cross-react with other species as described in the data sheet. AAA Biotech's LRPPRC can be used in a range of immunoassay formats including, but not limited to, Western Blot (WB). Researchers should empirically determine the suitability of the LRPPRC lrpprc 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. The amino acid sequence is listed below: Synthetic peptide located within the following region: NEYKFSPTDF LAKMEEANIQ PNRVTYQRLI ASYCNVGDIE GASKILGFMK. It is sometimes possible for the material contained within the vial of "LRPPRC, 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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