Protein Name: Leucine-rich PPR motif-containing protein, mitochondrial
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UniprotKB/SwissProt ID: LPPRC_HUMAN (P42704)
Gene Name: LRPPRC
Synonyms: LRP130
Organism: Homo sapiens (Human).
Function: 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).
Other Modifications: View all modification sites in dbPTM
Protein Subcellular Localization: Mitochondrion. Nucleus, nucleoplasm. Nucleus inner membrane. Nucleus outer membrane. Note=Seems to be predominantly mitochondrial.
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Protein disease:
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| Disease database |
Database Entry |
Disease information | | KEGG | H00072 | Pyruvate dehydrogenase complex deficiency, including: Leigh syndrome; Pyruvate dehydrogenase phospha | | KEGG | H00473 | Mitochondrial respiratory chain deficiencies (MRCD), including: Mitochondrial complex I deficiency ( | | OMIM | 220111 | LEIGH SYNDROME, FRENCH-CANADIAN TYPE; LSFC ;;CYTOCHROME c OXIDASE DEFICIENCY, FRENCH-CANADIA | | OMIM | 607544 | LEUCINE-RICH PPR MOTIF-CONTAINING PROTEIN; LRPPRC ;;LEUCINE-RICH PROTEIN, 130-KD; LRP130 | | HPRD | 06343 | Leigh syndrome, French-Canadian type |
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Graphical Visualization of S-nitrosylation Sites:
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| Overview of Protein S-nitrosylation Sites with Functional and Structural Information |  |
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