ID  P42704;
PN  Leucine-rich PPR motif-containing protein, mitochondrial;
GN  LRPPRC;
OS  9606;
SL  Nucleus Position: SL-0178;
SL  Nucleus Position: SL-0179;
SL  Nucleus Position: SL-0182;
SL  Nucleus Position: SL-0183;
SL  Comments: Mitochondrion. Nucleus, nucleoplasm. Nucleus inner membrane. Nucleus outer membrane. Note=Seems to be predominantly mitochondrial.
DR  UNIPROT: P42704;
DR  UNIPROT: A0PJE3;
DR  UNIPROT: A8K1V1;
DR  UNIPROT: Q53PC0;
DR  UNIPROT: Q53QN7;
DR  UNIPROT: Q6ZUD8;
DR  UNIPROT: Q7Z7A6;
DR  UNIPROT: Q96D84;
DR  Pfam: PF01535;
DR  Pfam: PF13812;
DR  Pfam: PF17177;
DR  PROSITE: PS51375;
DR  OMIM: 220111;
DR  OMIM: 607544;
DR  DisGeNET: 10128;
DE  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). {ECO:0000250, ECO:0000269|PubMed:11585913, ECO:0000269|PubMed:12832482, ECO:0000269|PubMed:15081402, ECO:0000269|PubMed:15139850, ECO:0000269|PubMed:15272088, ECO:0000269|PubMed:17050673}.
DE  Disease: Mitochondrial complex IV deficiency, nuclear type 5 (MC4DN5) [MIM:220111]: An autosomal recessive, severe mitochondrial disease with multisystemic manifestations and early onset. Clinical features include delayed psychomotor development, impaired intellectual development with speech delay, mild dysmorphic facial features, hypotonia, ataxia, and seizures. Brain imaging shows bilaterally symmetrical necrotic lesions in subcortical brain regions. Mortality is high, due to episodes of severe metabolic acidosis and coma. {ECO:0000269|PubMed:12529507, ECO:0000269|PubMed:26510951}. Note=The disease is caused by variants affecting the gene represented in this entry.
DE  Reference Proteome: Yes;
DE  Interaction: P04626; IntAct: EBI-8770853; Score: 0.53
DE  Interaction: Q9Y6K9; IntAct: EBI-361392; Score: 0.00
DE  Interaction: Q15653; IntAct: EBI-365313; Score: 0.00
DE  Interaction: P60709; IntAct: EBI-353790; Score: 0.40
DE  Interaction: O75365; IntAct: EBI-1060175; Score: 0.00
DE  Interaction: Q9Y5J5; IntAct: EBI-1060455; Score: 0.00
DE  Interaction: Q9P2S5; IntAct: EBI-1060482; Score: 0.00
DE  Interaction: P01106; IntAct: EBI-1237540; Score: 0.67
DE  Interaction: Q9Y4K3; IntAct: EBI-1065456; Score: 0.00
DE  Interaction: Q9HC98; IntAct: EBI-1066112; Score: 0.00
DE  Interaction: P60520; IntAct: EBI-1066983; Score: 0.00
DE  Interaction: P23508; IntAct: EBI-1068590; Score: 0.00
DE  Interaction: P78396; IntAct: EBI-1070328; Score: 0.00
DE  Interaction: P19532; IntAct: EBI-1071516; Score: 0.00
DE  Interaction: P43360; IntAct: EBI-1073949; Score: 0.00
DE  Interaction: Q9UBN6; IntAct: EBI-1074138; Score: 0.00
DE  Interaction: P62330; IntAct: EBI-1074333; Score: 0.00
DE  Interaction: Q14164; IntAct: EBI-1075130; Score: 0.00
DE  Interaction: P01889; IntAct: EBI-1076912; Score: 0.00
DE  Interaction: P11171; IntAct: EBI-1079980; Score: 0.00
DE  Interaction: Q9Y2Q3; IntAct: EBI-1083828; Score: 0.00
DE  Interaction: Q9Y5V3; IntAct: EBI-1084294; Score: 0.00
DE  Interaction: P63104; IntAct: EBI-7192962; Score: 0.40
DE  Interaction: O70343; IntAct: EBI-1371067; Score: 0.40
DE  Interaction: Q9UBK2; IntAct: EBI-1371136; Score: 0.52
DE  Interaction: Q8AZK7; IntAct: EBI-9632715; Score: 0.35
DE  Interaction: P06730; IntAct: EBI-8582156; Score: 0.58
DE  Interaction: O94966; IntAct: EBI-2511876; Score: 0.40
DE  Interaction: Q70CQ1; IntAct: EBI-2512828; Score: 0.40
DE  Interaction: Q9NXR7; IntAct: EBI-2514089; Score: 0.40
DE  Interaction: Q81ME0; IntAct: EBI-2830399; Score: 0.00
DE  Interaction: Q9Z381; IntAct: EBI-2865280; Score: 0.00
DE  Interaction: Q9BSB4; IntAct: EBI-3255690; Score: 0.35
DE  Interaction: Q14197; IntAct: EBI-7825470; Score: 0.35
DE  Interaction: Q6QDQ4; IntAct: EBI-5276631; Score: 0.35
DE  Interaction: Q15327; IntAct: EBI-5653419; Score: 0.00
DE  Interaction: Q9GZT3; IntAct: EBI-7503132; Score: 0.56
DE  Interaction: O75381; IntAct: EBI-5911953; Score: 0.35
DE  Interaction: O00716; IntAct: EBI-7600105; Score: 0.35
DE  Interaction: P0DOE9; IntAct: EBI-6138583; Score: 0.35
DE  Interaction: P05919; IntAct: EBI-6175288; Score: 0.46
DE  Interaction: P19320; IntAct: EBI-6189915; Score: 0.35
DE  Interaction: Q77M19; IntAct: EBI-6270503; Score: 0.35
DE  Interaction: Q5U5Q3; IntAct: EBI-8011718; Score: 0.35
DE  Interaction: O35658; IntAct: EBI-6393534; Score: 0.35
DE  Interaction: P21860; IntAct: EBI-8771060; Score: 0.35
DE  Interaction: P20963; IntAct: EBI-9512492; Score: 0.35
DE  Interaction: P19838; IntAct: EBI-11322719; Score: 0.35
DE  Interaction: P05412; IntAct: EBI-11324725; Score: 0.35
DE  Interaction: Q9Y6E7; IntAct: EBI-10715009; Score: 0.35
DE  Interaction: P03177; IntAct: EBI-11721652; Score: 0.35
DE  Interaction: P06428; IntAct: EBI-11722493; Score: 0.35
DE  Interaction: P0C739; IntAct: EBI-11725101; Score: 0.35
DE  Interaction: Q9DUG7; IntAct: EBI-11734217; Score: 0.35
DE  Interaction: A2APR8; IntAct: EBI-11001201; Score: 0.35
DE  Interaction: Q8VC57; IntAct: EBI-11009211; Score: 0.35
DE  Interaction: P09803; IntAct: EBI-11019737; Score: 0.35
DE  Interaction: P51149; IntAct: EBI-11050319; Score: 0.35
DE  Interaction: P47755; IntAct: EBI-11117728; Score: 0.35
DE  Interaction: Q9HBM1; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: Q14573; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: P28715; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: Q9NPL8; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: Q9BZQ6; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: J3KS15; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: A8K7Q2; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: Q9P2K8; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: J3QL56; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: Q6UB35; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: P38117; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: Q6PJT7; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: Q02224; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: B4DR80; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: P39748; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: P36776; IntAct: EBI-11137058; Score: 0.35
DE  Interaction: P42768; IntAct: EBI-11139448; Score: 0.35
DE  Interaction: Q96EB6; IntAct: EBI-11139448; Score: 0.35
DE  Interaction: Q14CZ7; IntAct: EBI-11426979; Score: 0.35
DE  Interaction: P63000; IntAct: EBI-12451898; Score: 0.51
DE  Interaction: Q8IWL3; IntAct: EBI-13943458; Score: 0.35
DE  Interaction: Q9NZI8; IntAct: EBI-13949271; Score: 0.35
DE  Interaction: O14829; IntAct: EBI-14024386; Score: 0.35
DE  Interaction: Q96KR7; IntAct: EBI-14027299; Score: 0.35
DE  Interaction: Q6IQ23; IntAct: EBI-16398399; Score: 0.35
DE  Interaction: P62136; IntAct: EBI-16370519; Score: 0.35
DE  Interaction: Q96EV8; IntAct: EBI-16749117; Score: 0.35
DE  Interaction: Q9NWT8; IntAct: EBI-16786806; Score: 0.27
DE  Interaction: P10809; IntAct: EBI-16790769; Score: 0.27
DE  Interaction: O14880; IntAct: EBI-16796475; Score: 0.27
DE  Interaction: Q15118; IntAct: EBI-16796954; Score: 0.27
DE  Interaction: Q9BVS5; IntAct: EBI-16802257; Score: 0.27
DE  Interaction: O95714; IntAct: EBI-16811848; Score: 0.35
DE  Interaction: P08559; IntAct: EBI-20306509; Score: 0.35
DE  Interaction: Q9H6K4; IntAct: EBI-21937258; Score: 0.35
DE  Interaction: Q15007; IntAct: EBI-20595349; Score: 0.35
DE  Interaction: Q9HCE5; IntAct: EBI-20595531; Score: 0.35
DE  Interaction: O96013; IntAct: EBI-26962273; Score: 0.35
DE  Interaction: Q5T7N2; IntAct: EBI-20993270; Score: 0.35
DE  Interaction: Q9H1C4; IntAct: EBI-21266770; Score: 0.35
DE  Interaction: O95183; IntAct: EBI-21267749; Score: 0.35
DE  Interaction: P05067; IntAct: EBI-21132574; Score: 0.58
DE  Interaction: Q5T9A4; IntAct: EBI-21981699; Score: 0.35
DE  Interaction: Q9NVI7; IntAct: EBI-21981790; Score: 0.35
DE  Interaction: Q9NTX7; IntAct: EBI-22117924; Score: 0.35
DE  Interaction: P28562; IntAct: EBI-25377052; Score: 0.35
DE  Interaction: Q16828; IntAct: EBI-25377680; Score: 0.35
DE  Interaction: P27361; IntAct: EBI-25386687; Score: 0.35
DE  Interaction: O15530; IntAct: EBI-25387801; Score: 0.35
DE  Interaction: O94776; IntAct: EBI-25389141; Score: 0.35
DE  Interaction: P08631; IntAct: EBI-25390400; Score: 0.35
DE  Interaction: Q13480; IntAct: EBI-25393000; Score: 0.35
DE  Interaction: Q15256; IntAct: EBI-25393868; Score: 0.35
DE  Interaction: O84648; IntAct: EBI-22303579; Score: 0.35
DE  Interaction: P0DOF2; IntAct: EBI-25603126; Score: 0.35
DE  Interaction: Q9BY14; IntAct: EBI-25504841; Score: 0.35
DE  Interaction: Q8NHP6; IntAct: EBI-25617558; Score: 0.35
DE  Interaction: Q5EP34; IntAct: EBI-25772822; Score: 0.35
DE  Interaction: Q09161; IntAct: EBI-26396507; Score: 0.35
DE  Interaction: Q53F19; IntAct: EBI-26396827; Score: 0.35
DE  Interaction: Q6ZNK6; IntAct: EBI-26453464; Score: 0.35
DE  Interaction: O14561; IntAct: EBI-26494947; Score: 0.40
DE  Interaction: Q96T52; IntAct: EBI-27049069; Score: 0.27
DE  Interaction: Q96LU5; IntAct: EBI-27049466; Score: 0.27
DE  Interaction: Q9H300; IntAct: EBI-27049982; Score: 0.27
DE  Interaction: P35226; IntAct: EBI-27108918; Score: 0.35
DE  Interaction: P36507; IntAct: EBI-28935019; Score: 0.35
DE  Interaction: Q05513; IntAct: EBI-28938998; Score: 0.35
DE  Interaction: Q8NI60; IntAct: EBI-28943519; Score: 0.35
DE  Interaction: Q9H5K3; IntAct: EBI-28948637; Score: 0.35
DE  Interaction: Q8NCK7; IntAct: EBI-27103180; Score: 0.35
DE  Interaction: Q86VG3; IntAct: EBI-28997277; Score: 0.35
DE  Interaction: Q8N612; IntAct: EBI-34574737; Score: 0.27
DE  Interaction: Q5W0V3; IntAct: EBI-34574999; Score: 0.27
DE  Interaction: Q86V87; IntAct: EBI-34575191; Score: 0.27
DE  Interaction: P12830; IntAct: EBI-34581511; Score: 0.35
GO  GO:0000794;
GO  GO:0005856;
GO  GO:0016020;
GO  GO:0005874;
GO  GO:0042645;
GO  GO:0005739;
GO  GO:0005637;
GO  GO:0005640;
GO  GO:0005654;
GO  GO:0005634;
GO  GO:0048471;
GO  GO:1990904;
GO  GO:0048487;
GO  GO:0008017;
GO  GO:0003730;
GO  GO:0003723;
GO  GO:0003697;
GO  GO:0031625;
GO  GO:0000957;
GO  GO:0047497;
GO  GO:0051028;
GO  GO:0000961;
GO  GO:0070129;
TP  Membrane Topology: Unknown; Source: UniProt - Sequence Analysis;
SQ  MAALLRSARWLLRAGAAPRLPLSLRLLPGGPGRLHAASYLPAARAGPVAGGLLSPARLYAIAAKEKDIQEESTFSSRKIS
SQ  NQFDWALMRLDLSVRRTGRIPKKLLQKVFNDTCRSGGLGGSHALLLLRSCGSLLPELKLEERTEFAHRIWDTLQKLGAVY
SQ  DVSHYNALLKVYLQNEYKFSPTDFLAKMEEANIQPNRVTYQRLIASYCNVGDIEGASKILGFMKTKDLPVTEAVFSALVT
SQ  GHARAGDMENAENILTVMRDAGIEPGPDTYLALLNAYAEKGDIDHVKQTLEKVEKSELHLMDRDLLQIIFSFSKAGYPQY
SQ  VSEILEKVTCERRYIPDAMNLILLLVTEKLEDVALQILLACPVSKEDGPSVFGSFFLQHCVTMNTPVEKLTDYCKKLKEV
SQ  QMHSFPLQFTLHCALLANKTDLAKALMKAVKEEGFPIRPHYFWPLLVGRRKEKNVQGIIEILKGMQELGVHPDQETYTDY
SQ  VIPCFDSVNSARAILQENGCLSDSDMFSQAGLRSEAANGNLDFVLSFLKSNTLPISLQSIRSSLLLGFRRSMNINLWSEI
SQ  TELLYKDGRYCQEPRGPTEAVGYFLYNLIDSMSDSEVQAKEEHLRQYFHQLEKMNVKIPENIYRGIRNLLESYHVPELIK
SQ  DAHLLVESKNLDFQKTVQLTSSELESTLETLKAENQPIRDVLKQLILVLCSEENMQKALELKAKYESDMVTGGYAALINL
SQ  CCRHDKVEDALNLKEEFDRLDSSAVLDTGKYVGLVRVLAKHGKLQDAINILKEMKEKDVLIKDTTALSFFHMLNGAALRG
SQ  EIETVKQLHEAIVTLGLAEPSTNISFPLVTVHLEKGDLSTALEVAIDCYEKYKVLPRIHDVLCKLVEKGETDLIQKAMDF
SQ  VSQEQGEMVMLYDLFFAFLQTGNYKEAKKIIETPGIRARSARLQWFCDRCVANNQVETLEKLVELTQKLFECDRDQMYYN
SQ  LLKLYKINGDWQRADAVWNKIQEENVIPREKTLRLLAEILREGNQEVPFDVPELWYEDEKHSLNSSSASTTEPDFQKDIL
SQ  IACRLNQKKGAYDIFLNAKEQNIVFNAETYSNLIKLLMSEDYFTQAMEVKAFAETHIKGFTLNDAANSRLIITQVRRDYL
SQ  KEAVTTLKTVLDQQQTPSRLAVTRVIQALAMKGDVENIEVVQKMLNGLEDSIGLSKMVFINNIALAQIKNNNIDAAIENI
SQ  ENMLTSENKVIEPQYFGLAYLFRKVIEEQLEPAVEKISIMAERLANQFAIYKPVTDFFLQLVDAGKVDDARALLQRCGAI
SQ  AEQTPILLLFLLRNSRKQGKASTVKSVLELIPELNEKEEAYNSLMKSYVSEKDVTSAKALYEHLTAKNTKLDDLFLKRYA
SQ  SLLKYAGEPVPFIEPPESFEFYAQQLRKLRENSS
//