LRPPRC sirna

LRPPRC siRNA (Human)

Cat. Num. AAA8231739

• Gene Names: LRPPRC; LSFC; GP130; LRP130; CLONE-23970
• Reactivity: Human
• Applications: RNA Interference (RNAi)
• Purity: > 97%
• Synonyms: LRPPRC; LRPPRC siRNA (Human); LRP130; Leucine-rich PPR motif-containing protein mitochondrial; 130 kDa leucine-rich protein; LRP 130; GP130; LRPPRC sirna

• Host: Synthetic
• Reactivity: Human
• Specificity: LRPPRC siRNA (Human) is a target-specific 19-23 nt siRNA oligo duplexes designed to knock down gene expression.
• Purity/Purification: > 97%
• Form/Format: Lyophilized powder
• Sequence Length: 1394

• Applicable Applications for LRPPRC sirna: RNA Interference (RNAi)
• Quality Control: Oligonucleotide synthesis is monitored base by base through trityl analysis to ensure appropriate coupling efficiency. The oligo is subsequently purified by affinity-solid phase extraction. The annealed RNA duplex is further analyzed by mass spectrometry to verify the exact composition of the duplex. Each lot is compared to the previous lot by mass spectrometry to ensure maximum lot-to-lot consistency.
• Directions for Use: We recommends transfection with 100 nM siRNA 48 to 72 hours prior to cell lysis. Before resuspending, briefly centrifuge the tube to ensure the lyophilized siRNA is at the bottom of the tube. Resuspend the siRNA oligos to an appropriate concentration with DEPC water. For each vial, suitable for 250 transfections in 24 well plate (20 pmol for each well).
• Components: We offer pre-designed sets of 3 different target-specific siRNA oligo duplexes of human LRPPRC gene. Each vial contains 5 nmol of lyophilized siRNA. The duplexes can be transfected individually or pooled together to achieve knockdown of the target gene, which is most commonly assessed by qPCR or western blot. Our siRNA oligos are also chemically modified (2'-OMe) at no extra charge for increased stability and enhanced knockdown in vitro and in vivo.
• Preparation and Storage: Shipped at 4 degree C. Store at -20 degree C for one year.

Related Product Information for LRPPRC sirna

siRNA to inhibit LRPPRC expression using RNA interference

NCBI and Uniprot Product Information

• NCBI GI #: 31621305
• NCBI GeneID: 10128
• NCBI Accession #: NP_573566.2
• NCBI GenBank Nucleotide #: NM_133259.3
• UniProt Accession #: P42704; Q53PC0; Q53QN7; Q6ZUD8; Q7Z7A6; Q96D84; A0PJE3; A8K1V1
• Molecular Weight: 157,905 Da
• NCBI Official Full Name: leucine-rich PPR motif-containing protein, mitochondrial
• NCBI Official 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
• Protein Family: Leucine-rich PPR motif-containing protein
• UniProt Gene Name: LRPPRC
• UniProt Synonym Gene Names: LRP130; LRP 130
• UniProt Entry Name: LPPRC_HUMAN

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; membrane; mitochondrion; perinuclear region of cytoplasm; nuclear inner membrane; ribonucleoprotein complex; nucleus

Molecular Function: actin filament binding; protein binding; RNA binding; microtubule binding; beta-tubulin binding; single-stranded DNA binding

Biological Process: mRNA transport; regulation of transcription, DNA-dependent; transcription, DNA-dependent; mitochondrion transport along microtubule

Disease: Leigh Syndrome, French Canadian Type