leucine-rich PPR motif-containing protein, mitochondrial; 130 kD leucine-rich protein; LRP 130; GP130 (LRPPRC, LRP130)
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Function
- may play a role in RNA metabolism in both nuclei & mitochondria.
- in the nucleus, binds to HNRPA1-associated poly(A)mRNAs & 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 & gluconeogenic genes & may regulate docking of PPARGC1A to transcription factors
- seems to be involved in the transcription regulation of the multidrug-related genes MDR1 & MVP
- part of a nuclear factor that binds to the invMED1 element of MDR1 & MVP gene promoters
- binds single-stranded DNA (putative)
- interacts with CECR2, HEBP2, MAP1S & UXT
- interacts with PPARGC1A
- interacts with FOXO1 (putative)
- component of mRNP complexes associated with HNRPA1
Structure
Compartment
- mitochondria. nucleus, nucleoplasm
- nuclear membrane
- seems to be predominantly mitochondrial
Expression
- expressed ubiquitously
- expression is highest in heart, skeletal muscle, kidney & liver > brain, non-mucosal colon, spleen & placenta > small intestine, thymus, lung & peripheral blood leukocytes
Pathology
- defects in LRPPRC are the cause of Leigh syndrome, French-Canadian type