Rabbit LRPPRC Polyclonal Antibody | anti-LRPPRC antibody

LRPPRC (Leucine-rich Pentatricopeptide Repeat Containing, LSFC, GP130, LRP130) (FITC)

Cat. Num. AAA6424926

• Gene Names: LRPPRC; LSFC; GP130; LRP130; CLONE-23970
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot
• Purity: Purified by affinity chromatography.
• Synonyms: LRPPRC; Polyclonal Antibody; LRPPRC (Leucine-rich Pentatricopeptide Repeat Containing; LSFC; GP130; LRP130) (FITC); anti-LRPPRC antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes LRPPRC. Species Crossreactivity: human, mouse, rat
• Purity/Purification: Purified by affinity chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein Isothiocyanate (FITC).
• Concentration: ~1mg/ml (varies by lot)

• Applicable Applications for anti-LRPPRC antibody: Western Blot (WB)
• Application Notes: WB: 1:500-1:2000; Applications are based on unconjugated antibody.
• Immunogen: Recombinant protein corresponding to human LRPPRC.
• Conjugate: FITC
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Western Blot (WB)

(Western blot analysis of extracts of various cell lines, using LRPPRC antibody.)

Product Categories/Family for anti-LRPPRC antibody

Antibodies; Lipoproteins

NCBI and Uniprot Product Information

• NCBI GI #: 156632706
• NCBI GeneID: 10128
• UniProt Accession #: P42704; Q53PC0; Q53QN7; Q6ZUD8; Q7Z7A6; Q96D84; A0PJE3; A8K1V1
• Molecular Weight: 1394
• 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; LRP 130; 130 kDa leucine-rich protein; leucine-rich PPR-motif containing; mitochondrial leucine-rich PPR motif-containing protein
• 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; 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