Q6PB66 (LPPRC_MOUSE) Reviewed, UniProtKB/Swiss-Prot

Names and origin

Protein names

Recommended name:
Leucine-rich PPR motif-containing protein, mitochondrial

Alternative name(s):
130 kDa leucine-rich protein
Short name=LRP 130
Short name=mLRP130

Gene names

Name:	Lrpprc
Synonyms:	Lrp130

Organism

Mus musculus (Mouse) [Reference proteome]

Taxonomic identifier

10090 [NCBI]

Taxonomic lineage

cellular organisms › Eukaryota › Opisthokonta › Metazoa › Eumetazoa › Bilateria › Deuterostomia › Chordata › Craniata › Vertebrata › Gnathostomata › Teleostomi › Euteleostomi › Sarcopterygii › Tetrapoda › Amniota › Mammalia › Theria › Eutheria › Euarchontoglires › Glires › Rodentia › Sciurognathi › Muroidea › Muridae › Murinae › Mus › Mus

Protein attributes

Sequence length	1392 AA.
Sequence status	Complete.
Sequence processing	The displayed sequence is further processed into a mature form.
Protein existence	Evidence at protein level

General annotation (Comments)

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 By similarity. Binds single-stranded DNA.
Subunit structure	Interacts with CECR2, HEBP2, MAP1S and UXT By similarity. Interacts with PPARGC1A By similarity. Interacts with FOXO1. Component of mRNP complexes associated with HNRPA1 By similarity. Ref.5
Subcellular location	Mitochondrion By similarity. Nucleus. Nucleus › nucleoplasm By similarity. Nucleus inner membrane By similarity. Nucleus outer membrane By similarity Ref.1.
Tissue specificity	Strongly expressed in heart, liver and kidney. Weakly expressed in brain, skeletal muscle and testes. Ref.1
Developmental stage	Expressed at embryonic stages E7, E11, E15 and E17 with a slight increase of levels during development. Ref.1
Sequence similarities	Contains 20 PPR (pentatricopeptide) repeats.
Sequence caution	The sequence AAH59862.1 differs from that shown. Reason: Erroneous initiation. The sequence BAB29082.2 differs from that shown. Reason: Erroneous translation. CTG leucine codon is translated as initiator methionine.

Ontologies

Keywords
Biological process	Transcription Transcription regulation Transport mRNA transport
Cellular component	Membrane Mitochondrion Nucleus
Domain	Repeat Transit peptide
Ligand	DNA-binding RNA-binding
PTM	Acetylation
Technical term	Complete proteome Reference proteome
Gene Ontology (GO)
Biological_process	mRNA transport Inferred from electronic annotation. Source: UniProtKB-KW negative regulation of mitochondrial RNA catabolic process Inferred from mutant phenotype PubMed 21880015PubMed 22045337. Source: MGI regulation of mitochondrial translation Inferred from mutant phenotype PubMed 22045337. Source: MGI regulation of transcription, DNA-dependent Inferred from electronic annotation. Source: UniProtKB-KW transcription, DNA-dependent Inferred from electronic annotation. Source: UniProtKB-KW
Cellular_component	condensed nuclear chromosome Inferred from sequence or structural similarity. Source: HGNC cytoskeleton Inferred from sequence or structural similarity. Source: HGNC microtubule Inferred from electronic annotation. Source: Compara mitochondrial nucleoid Inferred from electronic annotation. Source: Compara mitochondrion Inferred from direct assay PubMed 14651853. Source: UniProtKB nuclear inner membrane Inferred from electronic annotation. Source: UniProtKB-SubCell nuclear outer membrane Inferred from electronic annotation. Source: UniProtKB-SubCell nucleoplasm Inferred from electronic annotation. Source: UniProtKB-SubCell perinuclear region of cytoplasm Inferred from sequence or structural similarity. Source: HGNC ribonucleoprotein complex Inferred from direct assay PubMed 22045337. Source: MGI
Molecular_function	RNA binding Inferred from direct assay PubMed 21880015. Source: MGI beta-tubulin binding Inferred from sequence or structural similarity. Source: HGNC single-stranded DNA binding Inferred from direct assay Ref.1. Source: MGI
Complete GO annotation...

Binary interactions

With	Entry	#Exp.	IntAct	Notes
Foxo1	Q9R1E0	2	EBI-1371262,EBI-1371343
Ppargc1a	O70343	2	EBI-1371262,EBI-1371053

Sequence annotation (Features)

	Feature key	Position(s)	Length	Description	Graphical view	Feature identifier
Molecule processing
	Transit peptide	1 – 59	59	Mitochondrion Potential
	Chain	60 – 1392	1333	Leucine-rich PPR motif-containing protein, mitochondrial		PRO_0000295546
Regions
	Repeat	125 – 159	35	PPR 1
	Repeat	160 – 194	35	PPR 2
	Repeat	195 – 229	35	PPR 3
	Repeat	230 – 264	35	PPR 4
	Repeat	265 – 299	35	PPR 5
	Repeat	300 – 334	35	PPR 6
	Repeat	402 – 436	35	PPR 7
	Repeat	437 – 471	35	PPR 8
	Repeat	677 – 708	32	PPR 9
	Repeat	709 – 745	37	PPR 10
	Repeat	746 – 783	38	PPR 11
	Repeat	784 – 820	37	PPR 12
	Repeat	821 – 856	36	PPR 13
	Repeat	953 – 987	35	PPR 14
	Repeat	1030 – 1064	35	PPR 15
	Repeat	1065 – 1101	37	PPR 16
	Repeat	1102 – 1136	35	PPR 17
	Repeat	1137 – 1175	39	PPR 18
	Repeat	1176 – 1210	35	PPR 19
	Repeat	1315 – 1349	35	PPR 20
	Region	931 – 1050	120	RNA-binding
Amino acid modifications
	Modified residue	186	1	N6-acetyllysine By similarity
	Modified residue	291	1	N6-acetyllysine By similarity
	Modified residue	749	1	N6-acetyllysine By similarity
Experimental info
	Sequence conflict	35	1	S → F in BAB93528. Ref.1
	Sequence conflict	52	1	R → L in BAB93528. Ref.1
	Sequence conflict	67	1	D → V in BAB93528. Ref.1
	Sequence conflict	466	1	K → E in BAB93528. Ref.1

Sequences

	Sequence		Length	Mass (Da)
	Q6PB66 [UniParc]. Last modified July 24, 2007. Version 2. Checksum: CC774240FCDBB2A6	FASTA	1,392	156,615
	10 20 30 40 50 60 MAALLRPARW LLGAAAAPRL PLSLRLPAGV PGRLSSVVRV AAVGSRPAAG ERLSQARLYA 70 80 90 100 110 120 IVAEKRDLQE EPAPVRKNSS QFDWALMRLD NSVRRTGRIT KGLLQRVFES TCSSGSPGSN 130 140 150 160 170 180 QALLLLRSCG SLLPELSLAE RTEFAHKIWD KLQQLGVVYD VSHYNALLKV YLQNEYKFSP 190 200 210 220 230 240 TDFLAKMEGA NIQPNRVTYQ RLIAAYCNVG DIEGASKILG FMKTKDLPIT EAVFSALVTG 250 260 270 280 290 300 HARAGDMENA ENILTVMKQA GIEPGPDTYL ALLNAHAERG DIGQVRQILE KVEKSDHYFM 310 320 330 340 350 360 DRDFLQVIFS FSKAGYPQYV SEILEKITYE RRSIPDAMNL ILFLATEKLE DTAFQVLLAL 370 380 390 400 410 420 PLSKDESSDN FGSFFLRHCV TLDLPPEKLI DYCRRLRDAK LHSSSLQFTL HCALQANRTA 430 440 450 460 470 480 LAKAVMEALR EEGFPIRPHY FWPLLAGHQK TKNVQGIIDI LKIMNKVGVD PDQETYINYV 490 500 510 520 530 540 FPCFDSAQSV RAALQENECL LASSTFAQAE VKNEAINGNL QNILSFLESN TLPFSFSSLR 550 560 570 580 590 600 NSLILGFRRS MNIDLWSKIT ELLYKDERYC SKPPGPAEAV GYFLYNLIDS MSDSEVQAKE 610 620 630 640 650 660 ERLRQYFHQL QEMNVKVPEN IYKGICNLLN TYHVPELIKD IKVLVDREKV DSQKTSQVTS 670 680 690 700 710 720 SDLESTLEKL KAEGQPVGSA LKQLLLLLCS EENMQKALEV KAKYESDMVI GGYAALINLC 730 740 750 760 770 780 CRHDNAEDAW NLKQEVDRLD ASAILDTAKY VALVKVLGKH SRLQDAINIL KEMKEKDVVI 790 800 810 820 830 840 KDATVLSFFH ILNGAALRGE IETVKQLHEA IVTLGLAKPS SNISFPLVTV HLEKGDLPAA 850 860 870 880 890 900 LEASIACHKK YKVLPRIHDV LCKLVEKGET DLIQKAMDFV SQEQGEMTML YDLFFAFLQT 910 920 930 940 950 960 GNYKEAKKII ETPGIRARPT RLQWFCDRCI ASNQVEALEK LVELTEKLFE CDRDQMYYNL 970 980 990 1000 1010 1020 LKLYKISSDW QRADAAWTKM QEENIIPRER TLRLLAEILK TSNQEVPFDV PELWFGDDRP 1030 1040 1050 1060 1070 1080 SLSPSSRSAG EDVTEKTLLS NCKLKKSKDA YNIFLKAEKQ NVVFSSETYS TLIGLLLSKD 1090 1100 1110 1120 1130 1140 DFTQAMHVKD FAETHIKGFT LNDAANSLLI IRQVRRDYLK GALATLRAAL DLKQVPSQIA 1150 1160 1170 1180 1190 1200 VTRLIQALAL KGDVESIEAI QRMVAGLDTI GLSKMVFINN IALAQMKNNK LDAAIENIEH 1210 1220 1230 1240 1250 1260 LLASENQAIE PQYFGLSYLF RKVIEEQMEP ALEKLSIMSE RMANQFALYK PVTDLFLQLV 1270 1280 1290 1300 1310 1320 DSGKVDEARA LLERCGAIAE QSSLLSVFCL RTSQKPKKAP VLKTLLELIP ELRDNDKVYS 1330 1340 1350 1360 1370 1380 CSMKSYALDK DVASAKALYE YLTAKNLKLD DLFLKRYAAL LKDVGEPVPF PEPPESFAFY 1390 IKQLKEARES PS

References

	« Hide 'large scale' referencesShow 'large scale' references »
[1]	"LRP130, a protein containing nine pentatricopeptide repeat motifs, interacts with a single-stranded cytosine-rich sequence of mouse hypervariable minisatellite Pc-1." Tsuchiya N., Fukuda H., Sugimura T., Nagao M., Nakagama H. Eur. J. Biochem. 269:2927-2933(2002) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [MRNA], MASS SPECTROMETRY, DNA-BINDING, SUBCELLULAR LOCATION, TISSUE SPECIFICITY, DEVELOPMENTAL STAGE.
[2]	"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)." The MGC Project Team Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. Strain: C57BL/6. Tissue: Brain.
[3]	"The transcriptional landscape of the mammalian genome." Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J. , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y. Science 309:1559-1563(2005) [PubMed] [Europe PMC] [Abstract] Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 681-1392. Strain: C57BL/6J. Tissue: Head.
[4]	"LRP130, a single-stranded DNA/RNA-binding protein, localizes at the outer nuclear and endoplasmic reticulum membrane, and interacts with mRNA in vivo." Tsuchiya N., Fukuda H., Nakashima K., Nagao M., Sugimura T., Nakagama H. Biochem. Biophys. Res. Commun. 317:736-743(2004) [PubMed] [Europe PMC] [Abstract] Cited for: RNA-BINDING.
[5]	"Defects in energy homeostasis in Leigh syndrome French Canadian variant through PGC-1alpha/LRP130 complex." Cooper M.P., Qu L., Rohas L.M., Lin J., Yang W., Erdjument-Bromage H., Tempst P., Spiegelman B.M. Genes Dev. 20:2996-3009(2006) [PubMed] [Europe PMC] [Abstract] Cited for: INTERACTION WITH FOXO1.
+	Additional computationally mapped references.

Cross-references

Sequence databases
EMBL GenBank DDBJ	AB027124 mRNA. Translation: BAB93528.1. Sequence problems. BC059862 mRNA. Translation: AAH59862.1. Different initiation. AK013955 mRNA. Translation: BAB29082.2. Sequence problems.
IPI	IPI00420706.
RefSeq	NP_082509.2. NM_028233.2.
UniGene	Mm.217027.
3D structure databases
ProteinModelPortal	Q6PB66.
ModBase	Search...
Protein-protein interaction databases
IntAct	Q6PB66. 4 interactions.
MINT	MINT-1840300.
PTM databases
PhosphoSite	Q6PB66.
Proteomic databases
PaxDb	Q6PB66.
PRIDE	Q6PB66.
Protocols and materials databases
StructuralBiologyKnowledgebase	Search...
Genome annotation databases
Ensembl	ENSMUST00000112308; ENSMUSP00000107927; ENSMUSG00000024120.
GeneID	72416.
KEGG	mmu:72416.
UCSC	uc008dtc.1. mouse.
Organism-specific databases
CTD	10128.
MGI	MGI:1919666. Lrpprc.
Phylogenomic databases
eggNOG	NOG292283.
GeneTree	ENSGT00390000016775.
HOGENOM	HOG000113350.
HOVERGEN	HBG097314.
InParanoid	Q6PB66.
OMA	CVTMNTP.
OrthoDB	EOG4DR9BG.
Gene expression databases
Bgee	Q6PB66.
CleanEx	MM_LRPPRC.
Genevestigator	Q6PB66.
Family and domain databases
InterPro	IPR002885. Pentatricopeptide_repeat. [Graphical view]
Pfam	PF01535. PPR. 2 hits. PF13812. PPR_3. 2 hits. [Graphical view]
TIGRFAMs	TIGR00756. PPR. 2 hits.
PROSITE	PS51375. PPR. 13 hits. [Graphical view]
ProtoNet	Search...
Other
ChiTaRS	LRPPRC. mouse.
NextBio	336222.
SOURCE	Search...

Entry information

Entry name

LPPRC_MOUSE

Accession

Primary (citable) accession number: Q6PB66
Secondary accession number(s): Q8K4V0, Q9CRX4

Entry history

Integrated into UniProtKB/Swiss-Prot:	July 24, 2007
Last sequence update:	July 24, 2007
Last modified:	May 29, 2013
This is version 78 of the entry and version 2 of the sequence. [Complete history]

Entry status

Reviewed (UniProtKB/Swiss-Prot)

Annotation program

Chordata Protein Annotation Program