Leucine-rich PPR motif-containing protein, mitochondrial - Homo sapiens

Leucine-rich PPR motif-containing protein, mitochondrial (Homo sapiens)

FASTA TEXT XML

Subcellular Location Biology & Function Disease Associations Interactions Cross-References

General Information

Protein Information
Protein Name	Leucine-rich PPR motif-containing protein, mitochondrial
Accession Code	P42704
Gene	LRPPRC
Organism	Homo sapiens \| Human (Taxonomy: 9606)
Part of Reference Proteome?	Yes
Sequence (Length: 1394)
MAALLRSARWLLRAGAAPRLPLSLRLLPGGPGRLHAASYLPAARAGPVAGGLLSPARLYAIAAKEKDIQEESTFSSRKISNQFDWALMRLDLSVRRTGRIPKKLLQKVFNDTCRSGGLGGSHALLLLRSC GSLLPELKLEERTEFAHRIWDTLQKLGAVYDVSHYNALLKVYLQNEYKFSPTDFLAKMEEANIQPNRVTYQRLIASYCNVGDIEGASKILGFMKTKDLPVTEAVFSALVTGHARAGDMENAENILTVMRD AGIEPGPDTYLALLNAYAEKGDIDHVKQTLEKVEKSELHLMDRDLLQIIFSFSKAGYPQYVSEILEKVTCERRYIPDAMNLILLLVTEKLEDVALQILLACPVSKEDGPSVFGSFFLQHCVTMNTPVEKL TDYCKKLKEVQMHSFPLQFTLHCALLANKTDLAKALMKAVKEEGFPIRPHYFWPLLVGRRKEKNVQGIIEILKGMQELGVHPDQETYTDYVIPCFDSVNSARAILQENGCLSDSDMFSQAGLRSEAANGN LDFVLSFLKSNTLPISLQSIRSSLLLGFRRSMNINLWSEITELLYKDGRYCQEPRGPTEAVGYFLYNLIDSMSDSEVQAKEEHLRQYFHQLEKMNVKIPENIYRGIRNLLESYHVPELIKDAHLLVESKN LDFQKTVQLTSSELESTLETLKAENQPIRDVLKQLILVLCSEENMQKALELKAKYESDMVTGGYAALINLCCRHDKVEDALNLKEEFDRLDSSAVLDTGKYVGLVRVLAKHGKLQDAINILKEMKEKDVL IKDTTALSFFHMLNGAALRGEIETVKQLHEAIVTLGLAEPSTNISFPLVTVHLEKGDLSTALEVAIDCYEKYKVLPRIHDVLCKLVEKGETDLIQKAMDFVSQEQGEMVMLYDLFFAFLQTGNYKEAKKI IETPGIRARSARLQWFCDRCVANNQVETLEKLVELTQKLFECDRDQMYYNLLKLYKINGDWQRADAVWNKIQEENVIPREKTLRLLAEILREGNQEVPFDVPELWYEDEKHSLNSSSASTTEPDFQKDIL IACRLNQKKGAYDIFLNAKEQNIVFNAETYSNLIKLLMSEDYFTQAMEVKAFAETHIKGFTLNDAANSRLIITQVRRDYLKEAVTTLKTVLDQQQTPSRLAVTRVIQALAMKGDVENIEVVQKMLNGLED SIGLSKMVFINNIALAQIKNNNIDAAIENIENMLTSENKVIEPQYFGLAYLFRKVIEEQLEPAVEKISIMAERLANQFAIYKPVTDFFLQLVDAGKVDDARALLQRCGAIAEQTPILLLFLLRNSRKQGK ASTVKSVLELIPELNEKEEAYNSLMKSYVSEKDVTSAKALYEHLTAKNTKLDDLFLKRYASLLKYAGEPVPFIEPPESFEFYAQQLRKLRENSS

Subcellular Location

Description
Mitochondrion. Nucleus, nucleoplasm. Nucleus inner membrane. Nucleus outer membrane. Note=Seems to be predominantly mitochondrial.
Position in the Nuclear Envelope
Location	Location ID	Description
Nuclear Envelope	SL-0178	The nuclear envelope is a membrane system which surrounds the nucleoplasm of eukaryotic cells. It is composed of the nuclear lamina, nuclear pore complexes and two nuclear membranes. The space between the two membranes is called the nuclear intermembrane space.
Nuclear Inner Membrane	SL-0179	The inner membrane of the nucleus is the membrane which separates the nuclear matrix from the intermembrane space. In mammals, the inner nuclear membrane is associated with heterochromatin and the nuclear lamina.
Nuclear Membrane	SL-0182	The membrane surrounding the nucleus. This term is used when it is not known if the protein is found in or associated with the inner or outer nuclear membrane.
Nuclear Outer Membrane	SL-0183	The outer membrane of the nucleus is the membrane facing the cytoplasm. In mammals, the outer nuclear membrane is continuous in many places with the rough endoplasmic reticulum and is dotted with ribosomes.
Membrane Topology
Topology	Source	Annotation Type
Unknown	UniProt	Sequence Analysis
Assigned Ontology terms

Cellular Component	Condensed Nuclear Chromosome (GO:0000794) Cytoskeleton (GO:0005856) Membrane (GO:0016020) Microtubule (GO:0005874) Mitochondrial Nucleoid (GO:0042645) Mitochondrion (GO:0005739) Nuclear Inner Membrane (GO:0005637) Nuclear Outer Membrane (GO:0005640) Nucleoplasm (GO:0005654) Nucleus (GO:0005634) Perinuclear Region Of Cytoplasm (GO:0048471) Ribonucleoprotein Complex (GO:1990904)

Biology & Function

Description
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). {By Similarity, Experimental EvidencePubMed:11585913, Experimental EvidencePubMed:12832482, Experimental EvidencePubMed:15081402, Experimental EvidencePubMed:15139850, Experimental EvidencePubMed:15272088, Experimental EvidencePubMed:17050673}.
Assigned Ontology terms

Biological Process	Mitochondrial RNA Catabolic Process (GO:0000957) Mitochondrion Transport Along Microtubule (GO:0047497) MRNA Transport (GO:0051028) Negative Regulation Of Mitochondrial RNA Catabolic Process (GO:0000961) Regulation Of Mitochondrial Translation (GO:0070129)
Molecular Function	Beta-Tubulin Binding (GO:0048487) Microtubule Binding (GO:0008017) MRNA 3'-UTR Binding (GO:0003730) RNA Binding (GO:0003723) Single-Stranded DNA Binding (GO:0003697) Ubiquitin Protein Ligase Binding (GO:0031625)

Disease associations

Description
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. {Experimental EvidencePubMed:12529507, Experimental EvidencePubMed:26510951}. Note=The disease is caused by variants affecting the gene represented in this entry.
Database Associations
OMIM	220111 607544
DisGeNET	10128

Protein-Protein Interactions

Interactions with Nuclear Envelope proteins (3 interactors)

Partner (NucEnvDB)		IntAct	Confidence score
Q15256	Receptor-type tyrosine-protein phosphatase R	EBI-25393868	0.35
P04626	Receptor tyrosine-protein kinase erbB-2	EBI-8770853	0.53
Q9NZI8	Insulin-like growth factor 2 mRNA-binding protein 1	EBI-13949271	0.35
Interactions with other proteins (132 interactors)

Partner (UniProt)		IntAct	Confidence score
Q9Y6K9	NF-kappa-B essential modulator (NEMO) (FIP-3) (IkB kinase-associated protein 1) (IKKAP1) (Inhibitor of nuclear factor kappa-B kinase subunit gamma) (I-kappa-B kinase subunit gamma) (IKK-gamma) (IKKG) (IkB kinase subunit gamma) (NF-kappa-B essential modifier)	EBI-361392	0.00
Q15653	NF-kappa-B inhibitor beta (NF-kappa-BIB) (I-kappa-B-beta) (IkB-B) (IkB-beta) (IkappaBbeta) (Thyroid receptor-interacting protein 9) (TR-interacting protein 9) (TRIP-9)	EBI-365313	0.00
P60709	Actin, cytoplasmic 1 (EC 3.6.4.-) (Beta-actin) [Cleaved into: Actin, cytoplasmic 1, N-terminally processed]	EBI-353790	0.40
O75365	Protein tyrosine phosphatase type IVA 3 (EC 3.1.3.48) (PRL-R) (Protein-tyrosine phosphatase 4a3) (Protein-tyrosine phosphatase of regenerating liver 3) (PRL-3)	EBI-1060175	0.00
Q9Y5J5	Pleckstrin homology-like domain family A member 3 (TDAG51/Ipl homolog 1)	EBI-1060455	0.00
Q9P2S5	WD repeat-containing protein WRAP73 (WD repeat-containing protein 8) (WD repeat-containing protein antisense to TP73 gene)	EBI-1060482	0.00
P01106	Myc proto-oncogene protein (Class E basic helix-loop-helix protein 39) (bHLHe39) (Proto-oncogene c-Myc) (Transcription factor p64)	EBI-1237540	0.67
Q9Y4K3	TNF receptor-associated factor 6 (EC 2.3.2.27) (E3 ubiquitin-protein ligase TRAF6) (Interleukin-1 signal transducer) (RING finger protein 85) (RING-type E3 ubiquitin transferase TRAF6)	EBI-1065456	0.00
Q9HC98	Serine/threonine-protein kinase Nek6 (EC 2.7.11.1) (Never in mitosis A-related kinase 6) (NimA-related protein kinase 6) (Protein kinase SID6-1512)	EBI-1066112	0.00
P60520	Gamma-aminobutyric acid receptor-associated protein-like 2 (GABA(A) receptor-associated protein-like 2) (Ganglioside expression factor 2) (GEF-2) (General protein transport factor p16) (Golgi-associated ATPase enhancer of 16 kDa) (GATE-16) (MAP1 light chain 3-related protein)	EBI-1066983	0.00
P23508	Colorectal mutant cancer protein (Protein MCC)	EBI-1068590	0.00
P78396	Cyclin-A1	EBI-1070328	0.00
P19532	Transcription factor E3 (Class E basic helix-loop-helix protein 33) (bHLHe33)	EBI-1071516	0.00
P43360	Melanoma-associated antigen 6 (Cancer/testis antigen 1.6) (CT1.6) (MAGE-6 antigen) (MAGE3B antigen)	EBI-1073949	0.00
Q9UBN6	Tumor necrosis factor receptor superfamily member 10D (Decoy receptor 2) (DcR2) (TNF-related apoptosis-inducing ligand receptor 4) (TRAIL receptor 4) (TRAIL-R4) (TRAIL receptor with a truncated death domain) (CD antigen CD264)	EBI-1074138	0.00
P62330	ADP-ribosylation factor 6 (EC 3.6.5.2)	EBI-1074333	0.00
Q14164	Inhibitor of nuclear factor kappa-B kinase subunit epsilon (I-kappa-B kinase epsilon) (IKK-E) (IKK-epsilon) (IkBKE) (EC 2.7.11.10) (Inducible I kappa-B kinase) (IKK-i)	EBI-1075130	0.00
P01889	HLA class I histocompatibility antigen, B alpha chain (Human leukocyte antigen B) (HLA-B)	EBI-1076912	0.00
P11171	Protein 4.1 (P4.1) (4.1R) (Band 4.1) (EPB4.1) (Erythrocyte membrane protein band 4.1)	EBI-1079980	0.00
Q9Y2Q3	Glutathione S-transferase kappa 1 (EC 2.5.1.18) (GST 13-13) (GST class-kappa) (GSTK1-1) (hGSTK1) (Glutathione S-transferase subunit 13)	EBI-1083828	0.00
Q9Y5V3	Melanoma-associated antigen D1 (MAGE tumor antigen CCF) (MAGE-D1 antigen) (Neurotrophin receptor-interacting MAGE homolog)	EBI-1084294	0.00
P63104	14-3-3 protein zeta/delta (Protein kinase C inhibitor protein 1) (KCIP-1)	EBI-7192962	0.40
O70343	Peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1-alpha) (PPAR-gamma coactivator 1-alpha) (PPARGC-1-alpha)	EBI-1371067	0.40
Q9UBK2	Peroxisome proliferator-activated receptor gamma coactivator 1-alpha (PGC-1-alpha) (PPAR-gamma coactivator 1-alpha) (PPARGC-1-alpha) (Ligand effect modulator 6)	EBI-1371136	0.52
Q8AZK7	Epstein-Barr nuclear antigen leader protein (EBNA-LP) (EBV nuclear antigen leader protein) (Epstein-Barr nuclear antigen 5) (EBNA-5) (EBV nuclear antigen 5)	EBI-9632715	0.35
P06730	Eukaryotic translation initiation factor 4E (eIF-4E) (eIF4E) (eIF-4F 25 kDa subunit) (mRNA cap-binding protein)	EBI-8582156	0.58
O94966	Ubiquitin carboxyl-terminal hydrolase 19 (EC 3.4.19.12) (Deubiquitinating enzyme 19) (Ubiquitin thioesterase 19) (Ubiquitin-specific-processing protease 19) (Zinc finger MYND domain-containing protein 9)	EBI-2511876	0.40
Q70CQ1	Ubiquitin carboxyl-terminal hydrolase 49 (EC 3.4.19.12) (Deubiquitinating enzyme 49) (Ubiquitin thioesterase 49) (Ubiquitin-specific-processing protease 49)	EBI-2512828	0.40
Q9NXR7	BRISC and BRCA1-A complex member 2 (BRCA1-A complex subunit BRE) (BRCA1/BRCA2-containing complex subunit 45) (Brain and reproductive organ-expressed protein)	EBI-2514089	0.40
Q81ME0	Phosphopentomutase (EC 5.4.2.7) (Phosphodeoxyribomutase)	EBI-2830399	0.00
Q9Z381	ORF 77	EBI-2865280	0.00
Q9BSB4	Autophagy-related protein 101	EBI-3255690	0.35
Q14197	Peptidyl-tRNA hydrolase ICT1, mitochondrial (EC 3.1.1.29) (39S ribosomal protein L58, mitochondrial) (MRP-L58) (Digestion substraction 1) (DS-1) (Immature colon carcinoma transcript 1 protein) (Mitochondrial large ribosomal subunit protein mL62)	EBI-7825470	0.35
Q6QDQ4	Nucleoprotein (Nucleocapsid protein)	EBI-5276631	0.35
Q15327	Ankyrin repeat domain-containing protein 1 (Cardiac ankyrin repeat protein) (Cytokine-inducible gene C-193 protein) (Cytokine-inducible nuclear protein)	EBI-5653419	0.00
Q9GZT3	SRA stem-loop-interacting RNA-binding protein, mitochondrial	EBI-7503132	0.56
O75381	Peroxisomal membrane protein PEX14 (PTS1 receptor-docking protein) (Peroxin-14) (Peroxisomal membrane anchor protein PEX14)	EBI-5911953	0.35
O00716	Transcription factor E2F3 (E2F-3)	EBI-7600105	0.35
P0DOE9	Non-structural protein 1 (NS1) (Non-structural protein 1C)	EBI-6138583	0.35
P05919	Protein Vpu (U ORF protein) (Viral protein U)	EBI-6175288	0.46
P19320	Vascular cell adhesion protein 1 (V-CAM 1) (VCAM-1) (INCAM-100) (CD antigen CD106)	EBI-6189915	0.35
Q77M19	V protein	EBI-6270503	0.35
Q5U5Q3	RNA-binding E3 ubiquitin-protein ligase MEX3C (EC 2.3.2.27) (RING finger and KH domain-containing protein 2) (RING finger protein 194) (RING-type E3 ubiquitin transferase MEX3C)	EBI-8011718	0.35
O35658	Complement component 1 Q subcomponent-binding protein, mitochondrial (GC1q-R protein) (Glycoprotein gC1qBP) (C1qBP)	EBI-6393534	0.35
P21860	Receptor tyrosine-protein kinase erbB-3 (EC 2.7.10.1) (Proto-oncogene-like protein c-ErbB-3) (Tyrosine kinase-type cell surface receptor HER3)	EBI-8771060	0.35
P20963	T-cell surface glycoprotein CD3 zeta chain (T-cell receptor T3 zeta chain) (CD antigen CD247)	EBI-9512492	0.35
P19838	Nuclear factor NF-kappa-B p105 subunit (DNA-binding factor KBF1) (EBP-1) (Nuclear factor of kappa light polypeptide gene enhancer in B-cells 1) [Cleaved into: Nuclear factor NF-kappa-B p50 subunit]	EBI-11322719	0.35
P05412	Transcription factor Jun (Activator protein 1) (AP1) (Proto-oncogene c-Jun) (Transcription factor AP-1 subunit Jun) (V-jun avian sarcoma virus 17 oncogene homolog) (p39)	EBI-11324725	0.35
Q9Y6E7	NAD-dependent protein lipoamidase sirtuin-4, mitochondrial (EC 2.3.1.-) (NAD-dependent ADP-ribosyltransferase sirtuin-4) (EC 2.4.2.-) (NAD-dependent protein biotinylase sirtuin-4) (EC 2.3.1.-) (NAD-dependent protein deacetylase sirtuin-4) (EC 2.3.1.286) (Regulatory protein SIR2 homolog 4) (SIR2-like protein 4)	EBI-10715009	0.35
P03177	Thymidine kinase (EC 2.7.1.21)	EBI-11721652	0.35
P06428	Protein E6	EBI-11722493	0.35
P0C739	Protein BNLF2a	EBI-11725101	0.35
Q9DUG7	Large T antigen	EBI-11734217	0.35
A2APR8	Mitotic checkpoint serine/threonine-protein kinase BUB1	EBI-11001201	0.35
Q8VC57	BTB/POZ domain-containing protein KCTD5	EBI-11009211	0.35
P09803	Cadherin-1 (ARC-1) (Epithelial cadherin) (E-cadherin) (Uvomorulin) (CD antigen CD324) [Cleaved into: E-Cad/CTF1; E-Cad/CTF2; E-Cad/CTF3]	EBI-11019737	0.35
P51149	Ras-related protein Rab-7a (EC 3.6.5.2)	EBI-11050319	0.35
P47755	F-actin-capping protein subunit alpha-2 (CapZ alpha-2)	EBI-11117728	0.35
Q9HBM1	Kinetochore protein Spc25 (hSpc25)	EBI-11137058	0.35
Q14573	Inositol 1,4,5-trisphosphate receptor type 3 (IP3 receptor isoform 3) (IP3R 3) (InsP3R3) (Type 3 inositol 1,4,5-trisphosphate receptor) (Type 3 InsP3 receptor)	EBI-11137058	0.35
P28715	DNA excision repair protein ERCC-5 (EC 3.1.-.-) (DNA repair protein complementing XP-G cells) (Xeroderma pigmentosum group G-complementing protein)	EBI-11137058	0.35
Q9NPL8	Complex I assembly factor TIMMDC1, mitochondrial (Protein M5-14) (Translocase of inner mitochondrial membrane domain-containing protein 1) (TIMM domain containing-protein 1)	EBI-11137058	0.35
Q9BZQ6	ER degradation-enhancing alpha-mannosidase-like protein 3 (EC 3.2.1.113) (Alpha-1,2-mannosidase EDEM3)	EBI-11137058	0.35
J3KS15	Peptidyl-tRNA hydrolase ICT1, mitochondrial	EBI-11137058	0.35
A8K7Q2	Heat shock cognate 71 kDa protein (cDNA FLJ77848)	EBI-11137058	0.35
Q9P2K8	eIF-2-alpha kinase GCN2 (EC 2.7.11.1) (Eukaryotic translation initiation factor 2-alpha kinase 4) (GCN2-like protein)	EBI-11137058	0.35
J3QL56	Protein SCO1 homolog, mitochondrial	EBI-11137058	0.35
Q6UB35	Monofunctional C1-tetrahydrofolate synthase, mitochondrial (EC 6.3.4.3) (Formyltetrahydrofolate synthetase)	EBI-11137058	0.35
P38117	Electron transfer flavoprotein subunit beta (Beta-ETF)	EBI-11137058	0.35
Q6PJT7	Zinc finger CCCH domain-containing protein 14 (Mammalian suppressor of tau pathology-2) (MSUT-2) (Renal carcinoma antigen NY-REN-37)	EBI-11137058	0.35
Q02224	Centromere-associated protein E (Centromere protein E) (CENP-E) (Kinesin-7) (Kinesin-related protein CENPE)	EBI-11137058	0.35
B4DR80	Serine/threonine-protein kinase 24 (cDNA FLJ61159, highly similar to Serine/threonine-protein kinase 24)	EBI-11137058	0.35
P39748	Flap endonuclease 1 (FEN-1) (EC 3.1.-.-) (DNase IV) (Flap structure-specific endonuclease 1) (Maturation factor 1) (MF1) (hFEN-1)	EBI-11137058	0.35
P36776	Lon protease homolog, mitochondrial (EC 3.4.21.53) (LONHs) (Lon protease-like protein) (LONP) (Mitochondrial ATP-dependent protease Lon) (Serine protease 15)	EBI-11137058	0.35
P42768	Actin nucleation-promoting factor WAS (Wiskott-Aldrich syndrome protein) (WASp)	EBI-11139448	0.35
Q96EB6	NAD-dependent protein deacetylase sirtuin-1 (hSIRT1) (EC 2.3.1.286) (NAD-dependent protein deacylase sirtuin-1) (EC 2.3.1.-) (Regulatory protein SIR2 homolog 1) (SIR2-like protein 1) (hSIR2) [Cleaved into: SirtT1 75 kDa fragment (75SirT1)]	EBI-11139448	0.35
Q14CZ7	FAST kinase domain-containing protein 3, mitochondrial	EBI-11426979	0.35
P63000	Ras-related C3 botulinum toxin substrate 1 (EC 3.6.5.2) (Cell migration-inducing gene 5 protein) (Ras-like protein TC25) (p21-Rac1)	EBI-12451898	0.51
Q8IWL3	Iron-sulfur cluster co-chaperone protein HscB (DnaJ homolog subfamily C member 20) [Cleaved into: Iron-sulfur cluster co-chaperone protein HscB, cytoplasmic (C-HSC20); Iron-sulfur cluster co-chaperone protein HscB, mitochondrial]	EBI-13943458	0.35
O14829	Serine/threonine-protein phosphatase with EF-hands 1 (PPEF-1) (EC 3.1.3.16) (Protein phosphatase with EF calcium-binding domain) (PPEF) (Serine/threonine-protein phosphatase 7) (PP7)	EBI-14024386	0.35
Q96KR7	Phosphatase and actin regulator 3 (Scaffold-associated PP1-inhibiting protein) (Scapinin)	EBI-14027299	0.35
Q6IQ23	Pleckstrin homology domain-containing family A member 7 (PH domain-containing family A member 7)	EBI-16398399	0.35
P62136	Serine/threonine-protein phosphatase PP1-alpha catalytic subunit (PP-1A) (EC 3.1.3.16)	EBI-16370519	0.35
Q96EV8	Dysbindin (Biogenesis of lysosome-related organelles complex 1 subunit 8) (BLOC-1 subunit 8) (Dysbindin-1) (Dystrobrevin-binding protein 1) (Hermansky-Pudlak syndrome 7 protein) (HPS7 protein)	EBI-16749117	0.35
Q9NWT8	Aurora kinase A-interacting protein (AURKA-interacting protein) (28S ribosomal protein S38, mitochondrial) (MRP-S38) (Mitochondrial small ribosomal subunit protein mS38)	EBI-16786806	0.27
P10809	60 kDa heat shock protein, mitochondrial (EC 5.6.1.7) (60 kDa chaperonin) (Chaperonin 60) (CPN60) (Heat shock protein 60) (HSP-60) (Hsp60) (HuCHA60) (Mitochondrial matrix protein P1) (P60 lymphocyte protein)	EBI-16790769	0.27
O14880	Microsomal glutathione S-transferase 3 (Microsomal GST-3) (Glutathione peroxidase MGST3) (EC 1.11.1.-) (LTC4 synthase MGST3) (EC 4.4.1.20) (Microsomal glutathione S-transferase III) (Microsomal GST-III)	EBI-16796475	0.27
Q15118	[Pyruvate dehydrogenase (acetyl-transferring)] kinase isozyme 1, mitochondrial (EC 2.7.11.2) (Pyruvate dehydrogenase kinase isoform 1) (PDH kinase 1)	EBI-16796954	0.27
Q9BVS5	tRNA (adenine(58)-N(1))-methyltransferase, mitochondrial (EC 2.1.1.220) (mRNA methyladenosine-N(1)-methyltransferase) (EC 2.1.1.-)	EBI-16802257	0.27
O95714	E3 ubiquitin-protein ligase HERC2 (EC 2.3.2.26) (HECT domain and RCC1-like domain-containing protein 2) (HECT-type E3 ubiquitin transferase HERC2)	EBI-16811848	0.35
P08559	Pyruvate dehydrogenase E1 component subunit alpha, somatic form, mitochondrial (EC 1.2.4.1) (PDHE1-A type I)	EBI-20306509	0.35
Q9H6K4	Optic atrophy 3 protein	EBI-21937258	0.35
Q15007	Pre-mRNA-splicing regulator WTAP (Female-lethal(2)D homolog) (hFL(2)D) (WT1-associated protein) (Wilms tumor 1-associating protein)	EBI-20595349	0.35
Q9HCE5	N6-adenosine-methyltransferase non-catalytic subunit (Methyltransferase-like protein 14) (hMETTL14)	EBI-20595531	0.35
O96013	Serine/threonine-protein kinase PAK 4 (EC 2.7.11.1) (p21-activated kinase 4) (PAK-4)	EBI-26962273	0.35
Q5T7N2	LINE-1 type transposase domain-containing protein 1 (ES cell-associated protein 11)	EBI-20993270	0.35
Q9H1C4	Protein unc-93 homolog B1 (Unc-93B1) (hUNC93B1)	EBI-21266770	0.35
O95183	Vesicle-associated membrane protein 5 (VAMP-5) (Myobrevin)	EBI-21267749	0.35
P05067	Amyloid-beta precursor protein (APP) (ABPP) (APPI) (Alzheimer disease amyloid A4 protein homolog) (Alzheimer disease amyloid protein) (Amyloid precursor protein) (Amyloid-beta (A4) precursor protein) (Amyloid-beta A4 protein) (Cerebral vascular amyloid peptide) (CVAP) (PreA4) (Protease nexin-II) (PN-II) [Cleaved into: N-APP; Soluble APP-alpha (S-APP-alpha); Soluble APP-beta (S-APP-beta); C99 (Beta-secretase C-terminal fragment) (Beta-CTF); Amyloid-beta protein 42 (Abeta42) (Beta-APP42); Amyloid-beta protein 40 (Abeta40) (Beta-APP40); C83 (Alpha-secretase C-terminal fragment) (Alpha-CTF); P3(42); P3(40); C80; Gamma-secretase C-terminal fragment 59 (Amyloid intracellular domain 59) (AICD-59) (AID(59)) (Gamma-CTF(59)); Gamma-secretase C-terminal fragment 57 (Amyloid intracellular domain 57) (AICD-57) (AID(57)) (Gamma-CTF(57)); Gamma-secretase C-terminal fragment 50 (Amyloid intracellular domain 50) (AICD-50) (AID(50)) (Gamma-CTF(50)); C31]	EBI-21132574	0.58
Q5T9A4	ATPase family AAA domain-containing protein 3B (AAA-TOB3)	EBI-21981699	0.35
Q9NVI7	ATPase family AAA domain-containing protein 3A	EBI-21981790	0.35
Q9NTX7	E3 ubiquitin-protein ligase RNF146 (EC 2.3.2.27) (Dactylidin) (Iduna) (RING finger protein 146) (RING-type E3 ubiquitin transferase RNF146)	EBI-22117924	0.35
P28562	Dual specificity protein phosphatase 1 (EC 3.1.3.16) (EC 3.1.3.48) (Dual specificity protein phosphatase hVH1) (Mitogen-activated protein kinase phosphatase 1) (MAP kinase phosphatase 1) (MKP-1) (Protein-tyrosine phosphatase CL100)	EBI-25377052	0.35
Q16828	Dual specificity protein phosphatase 6 (EC 3.1.3.16) (EC 3.1.3.48) (Dual specificity protein phosphatase PYST1) (Mitogen-activated protein kinase phosphatase 3) (MAP kinase phosphatase 3) (MKP-3)	EBI-25377680	0.35
P27361	Mitogen-activated protein kinase 3 (MAP kinase 3) (MAPK 3) (EC 2.7.11.24) (ERT2) (Extracellular signal-regulated kinase 1) (ERK-1) (Insulin-stimulated MAP2 kinase) (MAP kinase isoform p44) (p44-MAPK) (Microtubule-associated protein 2 kinase) (p44-ERK1)	EBI-25386687	0.35
O15530	3-phosphoinositide-dependent protein kinase 1 (hPDK1) (EC 2.7.11.1)	EBI-25387801	0.35
O94776	Metastasis-associated protein MTA2 (Metastasis-associated 1-like 1) (MTA1-L1 protein) (p53 target protein in deacetylase complex)	EBI-25389141	0.35
P08631	Tyrosine-protein kinase HCK (EC 2.7.10.2) (Hematopoietic cell kinase) (Hemopoietic cell kinase) (p59-HCK/p60-HCK) (p59Hck) (p61Hck)	EBI-25390400	0.35
Q13480	GRB2-associated-binding protein 1 (GRB2-associated binder 1) (Growth factor receptor bound protein 2-associated protein 1)	EBI-25393000	0.35
O84648	Conserved membrane protein	EBI-22303579	0.35
P0DOF2	Matrix protein (Phosphoprotein M2)	EBI-25603126	0.35
Q9BY14	Testis-expressed protein 101 (Cell surface receptor NYD-SP8) (Scleroderma-associated autoantigen) (Spermatogenesis-related gene protein)	EBI-25504841	0.35
Q8NHP6	Motile sperm domain-containing protein 2	EBI-25617558	0.35
Q5EP34	Polymerase acidic protein (EC 3.1.-.-) (RNA-directed RNA polymerase subunit P2)	EBI-25772822	0.35
Q09161	Nuclear cap-binding protein subunit 1 (80 kDa nuclear cap-binding protein) (CBP80) (NCBP 80 kDa subunit)	EBI-26396507	0.35
Q53F19	Nuclear cap-binding protein subunit 3 (Protein ELG)	EBI-26396827	0.35
Q6ZNK6	TRAF-interacting protein with FHA domain-containing protein B (TIFA-like protein)	EBI-26453464	0.35
O14561	Acyl carrier protein, mitochondrial (ACP) (CI-SDAP) (NADH-ubiquinone oxidoreductase 9.6 kDa subunit)	EBI-26494947	0.40
Q96T52	Mitochondrial inner membrane protease subunit 2 (EC 3.4.21.-) (IMP2-like protein)	EBI-27049069	0.27
Q96LU5	Mitochondrial inner membrane protease subunit 1 (EC 3.4.21.-) (IMP1-like protein)	EBI-27049466	0.27
Q9H300	Presenilins-associated rhomboid-like protein, mitochondrial (EC 3.4.21.105) (Mitochondrial intramembrane cleaving protease PARL) [Cleaved into: P-beta (Pbeta)]	EBI-27049982	0.27
P35226	Polycomb complex protein BMI-1 (Polycomb group RING finger protein 4) (RING finger protein 51)	EBI-27108918	0.35
P36507	Dual specificity mitogen-activated protein kinase kinase 2 (MAP kinase kinase 2) (MAPKK 2) (EC 2.7.12.2) (ERK activator kinase 2) (MAPK/ERK kinase 2) (MEK 2)	EBI-28935019	0.35
Q05513	Protein kinase C zeta type (EC 2.7.11.13) (nPKC-zeta)	EBI-28938998	0.35
Q8NI60	Atypical kinase COQ8A, mitochondrial (EC 2.7.-.-) (Chaperone activity of bc1 complex-like) (Chaperone-ABC1-like) (Coenzyme Q protein 8A) (aarF domain-containing protein kinase 3)	EBI-28943519	0.35
Q9H5K3	Protein O-mannose kinase (POMK) (EC 2.7.1.183) (Protein kinase-like protein SgK196) (Sugen kinase 196)	EBI-28948637	0.35
Q8NCK7	Monocarboxylate transporter 11 (MCT 11) (Solute carrier family 16 member 11)	EBI-27103180	0.35
Q86VG3	Intraflagellar transport-associated protein (Protein HEPIS)	EBI-28997277	0.35
Q8N612	FHF complex subunit HOOK interacting protein 1B (FHIP1B) (FTS and Hook-interacting protein) (FHIP)	EBI-34574737	0.27
Q5W0V3	FHF complex subunit HOOK interacting protein 2A (FHIP2A)	EBI-34574999	0.27
Q86V87	FHF complex subunit HOOK interacting protein 2B (FHIP2B) (Retinoic acid-induced protein 16)	EBI-34575191	0.27
P12830	Cadherin-1 (CAM 120/80) (Epithelial cadherin) (E-cadherin) (Uvomorulin) (CD antigen CD324) [Cleaved into: E-Cad/CTF1; E-Cad/CTF2; E-Cad/CTF3]	EBI-34581511	0.35

Cross-References

Database	Links
UNIPROT	P42704 A0PJE3 A8K1V1 Q53PC0 Q53QN7 Q6ZUD8 Q7Z7A6 Q96D84
Pfam	PF01535 PF13812 PF17177
PROSITE	PS51375
OMIM	220111 607544
DisGeNET	10128

Leucine-rich PPR motif-containing protein, mitochondrial (Homo sapiens)

General Information

Protein Information

Subcellular Location

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Position in the Nuclear Envelope

Membrane Topology

Assigned Ontology terms

Biology & Function

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Assigned Ontology terms

Disease associations

Description

Database Associations

Protein-Protein Interactions

Interactions with Nuclear Envelope proteins (3 interactors)

Interactions with other proteins (132 interactors)

Cross-References

Database

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Description

Description