Product Name
LRPPRC, Polyclonal Antibody
Full Product Name
LRPPRC Antibody, FITC conjugated
Product Synonym Names
Leucine-rich PPR motif-containing protein, mitochondrial; 130 kDa leucine-rich protein; LRP 130; GP130; LRPPRC; LRP130
Product Gene Name
anti-LRPPRC antibody
[Similar Products]
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
3D Structure
ModBase 3D Structure for P42704
Purity/Purification
>95%, Protein G purified
Immunogen
Recombinant Human Leucine-rich PPR motif-containing protein, mitochondrial protein (901-1036AA)
Buffer
Preservative: 0.03% Proclin 300
Constituents: 50% Glycerol, 0.01M PBS, pH 7.4
Preparation and Storage
Upon receipt, store at -20 degree C or -80 degree C. Avoid repeated freeze.
ISO Certification
Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.
Other Notes
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.
Related Product Information for
anti-LRPPRC antibody
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).
Product Categories/Family for anti-LRPPRC antibody
Epigenetics and Nuclear Signaling; Neuroscience
Applications Tested/Suitable for anti-LRPPRC antibody
ELISA (EIA)
NCBI/Uniprot data below describe general gene information for LRPPRC. It may not necessarily be applicable to this product.
NCBI Accession #
NP_573566.2
[Other Products]
NCBI GenBank Nucleotide #
NM_133259.3
[Other Products]
UniProt Primary Accession #
P42704
[Other Products]
UniProt Secondary Accession #
Q53PC0; Q53QN7; Q6ZUD8; Q7Z7A6; Q96D84; A0PJE3; A8K1V1[Other Products]
UniProt Related Accession #
P42704[Other Products]
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 [Similar Products]
NCBI Official Synonym Symbols
LSFC; GP130; LRP130; CLONE-23970
[Similar Products]
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 [Similar Products]
UniProt Synonym Gene Names
LRP130; LRP 130 [Similar Products]
NCBI Summary for LRPPRC
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 Comments for 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 ().
Research Articles on LRPPRC
1. To investigate the impact of the OXPHOS defect in the liver, aothors analyzed the mitochondrial phenotype in mice harboring an hepatocyte-specific inactivation of Lrpprc. Loss of LRPPRC in the liver caused a generalized growth delay, and typical histological features of mitochondrial hepatopathy.
Precautions
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