LRPPRC, Polyclonal Antibody

Leucine-rich PPR motif-containing protein; mitochondrial; 130 kDa leucine-rich protein; LRP 130; GP130; LRPPRC; LRP130

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

Ig

Rabbit

This LRPPRC antibody is generated from a rabbit immunized with a KLH conjugated synthetic peptide between 1203-1236 amino acids from human LRPPRC.

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Small volumes of anti-LRPPRC antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

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 (By similarity).

New Antibodies; Primary Antibodies; Neuroscience

Western Blot (WB), ELISA (EIA)

WB:~~1:2000

Anti-LRPPRC Antibody (C-Term) at 1:2000 dilution + HepG2 whole cell lysate
Lysates/proteins at 20 ug per lane.
Secondary
Goat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution.
Predicted band size : 158 kDa
Blocking/Dilution buffer: 5% NFDM/TBST.

157905 Da

leucine rich pentatricopeptide repeat containing

leucine-rich PPR motif-containing protein, mitochondrial

Leucine-rich PPR motif-containing protein, mitochondrial

LRP130; LRP 130  [Similar Products]

LPPRC_HUMAN

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]

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: condensed nuclear chromosome; cytoskeleton; membrane; microtubule; mitochondrion; nuclear inner membrane; nuclear outer membrane; nucleoplasm; nucleus; perinuclear region of cytoplasm; ribonucleoprotein complex

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

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

Disease: Leigh Syndrome, French Canadian Type

Xu F.,et al.Biochem. J. 382:331-336 (2004). Ota T.,et al.Nat. Genet. 36:40-45 (2004). Hillier L.W.,et al.Nature 434:724-731 (2005). Hou J.,et al.In Vitro Cell. Dev. Biol. Anim. 30A:111-114 (1994). Bienvenut W.V.,et al.Submitted (JUL-2007) to UniProtKB.

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