Anti-LRPPRC/GP130 Antibody Picoband® Fluoro647 Conjugated

Leucine-rich PPR motif-containing protein, mitochondrial is a protein that in humans is encoded by the LRPPRC gene. It is mapped to 2p21. 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.

Expressed ubiquitously. Expression is highest in heart, skeletal muscle, kidney and liver, intermediate in brain, non-mucosal colon, spleen and placenta, and lowest in small intestine, thymus, lung and peripheral blood leukocytes.

Cancer, Cancer Metabolism, Energy Metabolism, Energy Transfer Pathways, Integration Of Energy, Integration Of Energy Metabolism, Metabolic Signaling Pathway, Metabolic Signaling Pathways, Metabolism, Mitochondrial, Mitochondrial Markers, Mitochondrial Metabolism, Pathways and Processes, Signal Transduction

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.

1. Harmel, J., Ruzzenente, B., Terzioglu, M., Spahr, H., Falkenberg, M., Larsson, N.-G. The leucine-rich pentatricopeptide repeat-containing protein (LRPPRC) does not activate transcription in mammalian mitochondria. J. Biol. Chem. 288: 15510-15519, 2013. 2. Hou, J., Wang, F., McKeehan, W. L. Molecular cloning and expression of the gene for a major leucine-rich protein from human hepatoblastoma cells (HepG2) . In Vitro Cell Dev. Biol. Anim. 30A: 111-114, 1994. 3. Liu, L., McKeehan, W. L. Sequence analysis of LRPPRC and its SEC1 domain interaction partners suggest roles in cytoskeletal organization, vesicular trafficking, nucleocytosolic shuttling, and chromosome activity. Genomics 79: 124-136, 2002.