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Protein

Leucine-rich PPR motif-containing protein, mitochondrial

Gene

LRPPRC

Organism
Homo sapiens (Human)
Status
Reviewed-Annotation score:

Annotation score:5 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
-Experimental evidence at protein leveli <p>This indicates the type of evidence that supports the existence of the protein. Note that the ‘protein existence’ evidence does not give information on the accuracy or correctness of the sequence(s) displayed.<p><a href='/help/protein_existence' target='_top'>More...</a></p>

<p>This section provides any useful information about the protein, mostly biological knowledge.<p><a href='/help/function_section' target='_top'>More...</a></p>Functioni

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 similarity6 Publications

<p>The <a href="http://www.geneontology.org/">Gene Ontology (GO)</a> project provides a set of hierarchical controlled vocabulary split into 3 categories:<p><a href='/help/gene_ontology' target='_top'>More...</a></p>GO - Molecular functioni

  • beta-tubulin binding Source: HGNC
  • microtubule binding Source: HGNC
  • RNA binding Source: UniProtKB
  • single-stranded DNA binding Source: Ensembl
  • ubiquitin protein ligase binding Source: ParkinsonsUK-UCL

GO - Biological processi

<p>UniProtKB Keywords constitute a <a href="http://www.uniprot.org/keywords">controlled vocabulary</a> with a hierarchical structure. Keywords summarise the content of a UniProtKB entry and facilitate the search for proteins of interest.<p><a href='/help/keywords' target='_top'>More...</a></p>Keywordsi

Molecular functionDNA-binding, RNA-binding
Biological processmRNA transport, Transcription, Transcription regulation, Transport

Enzyme and pathway databases

Reactome - a knowledgebase of biological pathways and processes

More...
Reactomei
R-HSA-5628897 TP53 Regulates Metabolic Genes
R-HSA-611105 Respiratory electron transport

<p>This section provides information about the protein and gene name(s) and synonym(s) and about the organism that is the source of the protein sequence.<p><a href='/help/names_and_taxonomy_section' target='_top'>More...</a></p>Names & Taxonomyi

<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides an exhaustive list of all names of the protein, from commonly used to obsolete, to allow unambiguous identification of a protein.<p><a href='/help/protein_names' target='_top'>More...</a></p>Protein namesi
Recommended name:
Leucine-rich PPR motif-containing protein, mitochondrial
Alternative name(s):
130 kDa leucine-rich protein
Short name:
LRP 130
GP130
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section indicates the name(s) of the gene(s) that code for the protein sequence(s) described in the entry. Four distinct tokens exist: ‘Name’, ‘Synonyms’, ‘Ordered locus names’ and ‘ORF names’.<p><a href='/help/gene_name' target='_top'>More...</a></p>Gene namesi
Name:LRPPRC
Synonyms:LRP130
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section provides information on the name(s) of the organism that is the source of the protein sequence.<p><a href='/help/organism-name' target='_top'>More...</a></p>OrganismiHomo sapiens (Human)
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section shows the unique identifier assigned by the NCBI to the source organism of the protein. This is known as the ‘taxonomic identifier’ or ‘taxid’.<p><a href='/help/taxonomic_identifier' target='_top'>More...</a></p>Taxonomic identifieri9606 [NCBI]
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section contains the taxonomic hierarchical classification lineage of the source organism. It lists the nodes as they appear top-down in the taxonomic tree, with the more general grouping listed first.<p><a href='/help/taxonomic_lineage' target='_top'>More...</a></p>Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
<p>This subsection of the <a href="http://www.uniprot.org/help/names_and_taxonomy_section">Names and taxonomy</a> section is present for entries that are part of a <a href="http://www.uniprot.org/proteomes">proteome</a>, i.e. of a set of proteins thought to be expressed by organisms whose genomes have been completely sequenced.<p><a href='/help/proteomes_manual' target='_top'>More...</a></p>Proteomesi
  • UP000005640 <p>A UniProt <a href="http://www.uniprot.org/manual/proteomes_manual">proteome</a> can consist of several components. <br></br>The component name refers to the genomic component encoding a set of proteins.<p><a href='/help/proteome_component' target='_top'>More...</a></p> Componenti: Chromosome 2

Organism-specific databases

Eukaryotic Pathogen Database Resources

More...
EuPathDBi
HostDB:ENSG00000138095.18

Human Gene Nomenclature Database

More...
HGNCi
HGNC:15714 LRPPRC

Online Mendelian Inheritance in Man (OMIM)

More...
MIMi
607544 gene

neXtProt; the human protein knowledge platform

More...
neXtProti
NX_P42704

<p>This section provides information on the location and the topology of the mature protein in the cell.<p><a href='/help/subcellular_location_section' target='_top'>More...</a></p>Subcellular locationi

Extracellular region or secreted Cytosol Plasma membrane Cytoskeleton Lysosome Endosome Peroxisome ER Golgi apparatus Nucleus Mitochondrion Manual annotation Automatic computational assertionGraphics by Christian Stolte; Source: COMPARTMENTS

Keywords - Cellular componenti

Membrane, Mitochondrion, Nucleus

<p>This section provides information on the disease(s) and phenotype(s) associated with a protein.<p><a href='/help/pathology_and_biotech_section' target='_top'>More...</a></p>Pathology & Biotechi

<p>This subsection of the ‘Pathology and Biotech’ section provides information on the disease(s) associated with genetic variations in a given protein. The information is extracted from the scientific literature and diseases that are also described in the <a href="http://www.ncbi.nlm.nih.gov/sites/entrez?db=omim">OMIM</a> database are represented with a <a href="http://www.uniprot.org/diseases">controlled vocabulary</a> in the following way:<p><a href='/help/involvement_in_disease' target='_top'>More...</a></p>Involvement in diseasei

Leigh syndrome French-Canadian type (LSFC)2 Publications
The disease is caused by mutations affecting the gene represented in this entry.
Disease descriptionSevere neurological disorder characterized by bilaterally symmetrical necrotic lesions in subcortical brain regions that is commonly associated with systemic cytochrome c oxidase (COX) deficiency. In the Saguenay-Lac Saint Jean region of Quebec province in Canada, a biochemically distinct form of Leigh syndrome with COX deficiency has been described. Patients have been observed to have a developmental delay, hypotonia, mild facial dysmorphism, chronic well-compensated metabolic acidosis, and high mortality due to episodes of severe acidosis and coma. Enzyme activity was close to normal in kidney and heart, 50% of normal in fibroblasts and skeletal muscle, and nearly absent in brain and liver. LSFC patients show reduced (<30%) levels of LRPPRC in both fibroblast and liver mitochondria and a specifically reduced translation of COX subunits MT-CO1/COXI and MT-CO3 (COXIII).
See also OMIM:220111
Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section describes natural variant(s) of the protein sequence.<p><a href='/help/variant' target='_top'>More...</a></p>Natural variantiVAR_018656354A → V in LSFC. 1 PublicationCorresponds to variant dbSNP:rs119466000EnsemblClinVar.1
Natural variantiVAR_075428866Missing in LSFC; unknown pathological significance. 1 Publication1
Natural variantiVAR_075429909Missing in LSFC; unknown pathological significance. 1 Publication1

Keywords - Diseasei

Disease mutation, Leigh syndrome, Primary mitochondrial disease

Organism-specific databases

DisGeNET

More...
DisGeNETi
10128

MalaCards human disease database

More...
MalaCardsi
LRPPRC
MIMi220111 phenotype

Open Targets

More...
OpenTargetsi
ENSG00000138095

Orphanet; a database dedicated to information on rare diseases and orphan drugs

More...
Orphaneti
70472 Congenital lactic acidosis, Saguenay-Lac-Saint-Jean type

The Pharmacogenetics and Pharmacogenomics Knowledge Base

More...
PharmGKBi
PA30459

Polymorphism and mutation databases

BioMuta curated single-nucleotide variation and disease association database

More...
BioMutai
LRPPRC

Domain mapping of disease mutations (DMDM)

More...
DMDMi
156632706

<p>This section describes post-translational modifications (PTMs) and/or processing events.<p><a href='/help/ptm_processing_section' target='_top'>More...</a></p>PTM / Processingi

Molecule processing

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a transit peptide.<p><a href='/help/transit' target='_top'>More...</a></p>Transit peptidei1 – 59MitochondrionSequence analysisAdd BLAST59
<p>This subsection of the ‘PTM / Processing’ section describes the extent of a polypeptide chain in the mature protein following processing.<p><a href='/help/chain' target='_top'>More...</a></p>ChainiPRO_000008446760 – 1394Leucine-rich PPR motif-containing protein, mitochondrialAdd BLAST1335

Amino acid modifications

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘PTM / Processing’ section specifies the position and type of each modified residue excluding <a href="http://www.uniprot.org/manual/lipid">lipids</a>, <a href="http://www.uniprot.org/manual/carbohyd">glycans</a> and <a href="http://www.uniprot.org/manual/crosslnk">protein cross-links</a>.<p><a href='/help/mod_res' target='_top'>More...</a></p>Modified residuei155N6-acetyllysineCombined sources1
Modified residuei187N6-acetyllysineCombined sources1
Modified residuei226N6-acetyllysineBy similarity1
Modified residuei292N6-acetyllysineCombined sources1
Modified residuei463N6-acetyllysineBy similarity1
Modified residuei613N6-acetyllysineCombined sources1
Modified residuei726N6-acetyllysineCombined sources1
Modified residuei750N6-acetyllysineCombined sources1
Modified residuei1026PhosphoserineCombined sources1
Modified residuei1027PhosphoserineBy similarity1
Modified residuei1029PhosphoserineCombined sources1
Modified residuei1136PhosphothreonineCombined sources1
Modified residuei1138PhosphoserineCombined sources1

Keywords - PTMi

Acetylation, Phosphoprotein

Proteomic databases

Encyclopedia of Proteome Dynamics

More...
EPDi
P42704

MaxQB - The MaxQuant DataBase

More...
MaxQBi
P42704

PaxDb, a database of protein abundance averages across all three domains of life

More...
PaxDbi
P42704

PeptideAtlas

More...
PeptideAtlasi
P42704

PRoteomics IDEntifications database

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PRIDEi
P42704

ProteomicsDB human proteome resource

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ProteomicsDBi
55547

PTM databases

CarbonylDB database of protein carbonylation sites

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CarbonylDBi
P42704

iPTMnet integrated resource for PTMs in systems biology context

More...
iPTMneti
P42704

Comprehensive resource for the study of protein post-translational modifications (PTMs) in human, mouse and rat.

More...
PhosphoSitePlusi
P42704

SwissPalm database of S-palmitoylation events

More...
SwissPalmi
P42704

<p>This section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms.<p><a href='/help/expression_section' target='_top'>More...</a></p>Expressioni

<p>This subsection of the ‘Expression’ section provides information on the expression of a gene at the mRNA or protein level in cells or in tissues of multicellular organisms. By default, the information is derived from experiments at the mRNA level, unless specified ‘at protein level’. <br></br>Examples: <a href="http://www.uniprot.org/uniprot/P92958#expression">P92958</a>, <a href="http://www.uniprot.org/uniprot/Q8TDN4#expression">Q8TDN4</a>, <a href="http://www.uniprot.org/uniprot/O14734#expression">O14734</a><p><a href='/help/tissue_specificity' target='_top'>More...</a></p>Tissue specificityi

Expressed ubiquitously. Expression is highest in heart, skeletal muscle, kidney and liver, intermediate in brain, non-mucosal colon, spleen and placenta, and lowest in small intestine, thymus, lung and peripheral blood leukocytes.2 Publications

Gene expression databases

Bgee dataBase for Gene Expression Evolution

More...
Bgeei
ENSG00000138095 Expressed in 242 organ(s), highest expression level in skeletal muscle tissue of rectus abdominis

CleanEx database of gene expression profiles

More...
CleanExi
HS_LRPPRC

ExpressionAtlas, Differential and Baseline Expression

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ExpressionAtlasi
P42704 baseline and differential

Genevisible search portal to normalized and curated expression data from Genevestigator

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Genevisiblei
P42704 HS

Organism-specific databases

Human Protein Atlas

More...
HPAi
CAB033786
HPA036408
HPA036409

<p>This section provides information on the quaternary structure of a protein and on interaction(s) with other proteins or protein complexes.<p><a href='/help/interaction_section' target='_top'>More...</a></p>Interactioni

<p>This subsection of the <a href="http://www.uniprot.org/help/interaction_section">'Interaction'</a> section provides information about the protein quaternary structure and interaction(s) with other proteins or protein complexes (with the exception of physiological receptor-ligand interactions which are annotated in the <a href="http://www.uniprot.org/help/function_section">'Function'</a> section).<p><a href='/help/subunit_structure' target='_top'>More...</a></p>Subunit structurei

Interacts with CECR2, HEBP2, MAP1S and UXT. Interacts with PPARGC1A. Interacts with FOXO1 (By similarity). Component of mRNP complexes associated with HNRPA1.By similarity4 Publications

<p>This subsection of the '<a href="http://www.uniprot.org/help/interaction_section%27">Interaction</a> section provides information about binary protein-protein interactions. The data presented in this section are a quality-filtered subset of binary interactions automatically derived from the <a href="http://www.ebi.ac.uk/intact/">IntAct database</a>. It is updated on a monthly basis. Each binary interaction is displayed on a separate line.<p><a href='/help/binary_interactions' target='_top'>More...</a></p>Binary interactionsi

GO - Molecular functioni

Protein-protein interaction databases

The Biological General Repository for Interaction Datasets (BioGrid)

More...
BioGridi
115432, 193 interactors

CORUM comprehensive resource of mammalian protein complexes

More...
CORUMi
P42704

Database of interacting proteins

More...
DIPi
DIP-27543N

Protein interaction database and analysis system

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IntActi
P42704, 77 interactors

Molecular INTeraction database

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MINTi
P42704

STRING: functional protein association networks

More...
STRINGi
9606.ENSP00000260665

<p>This section provides information on the tertiary and secondary structure of a protein.<p><a href='/help/structure_section' target='_top'>More...</a></p>Structurei

3D structure databases

Protein Model Portal of the PSI-Nature Structural Biology Knowledgebase

More...
ProteinModelPortali
P42704

Database of comparative protein structure models

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ModBasei
Search...

MobiDB: a database of protein disorder and mobility annotations

More...
MobiDBi
Search...

<p>This section provides information on sequence similarities with other proteins and the domain(s) present in a protein.<p><a href='/help/family_and_domains_section' target='_top'>More...</a></p>Family & Domainsi

Domains and Repeats

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section indicates the positions and types of repeated sequence motifs or repeated domains within the protein.<p><a href='/help/repeat' target='_top'>More...</a></p>Repeati126 – 160PPR 1Add BLAST35
Repeati161 – 195PPR 2Add BLAST35
Repeati196 – 230PPR 3Add BLAST35
Repeati231 – 265PPR 4Add BLAST35
Repeati266 – 300PPR 5Add BLAST35
Repeati301 – 335PPR 6Add BLAST35
Repeati403 – 437PPR 7Add BLAST35
Repeati438 – 472PPR 8Add BLAST35
Repeati678 – 709PPR 9Add BLAST32
Repeati710 – 746PPR 10Add BLAST37
Repeati747 – 784PPR 11Add BLAST38
Repeati785 – 820PPR 12Add BLAST36
Repeati821 – 856PPR 13Add BLAST36
Repeati954 – 988PPR 14Add BLAST35
Repeati1031 – 1065PPR 15Add BLAST35
Repeati1066 – 1102PPR 16Add BLAST37
Repeati1103 – 1137PPR 17Add BLAST35
Repeati1138 – 1175PPR 18Add BLAST38
Repeati1176 – 1210PPR 19Add BLAST35
Repeati1317 – 1351PPR 20Add BLAST35

Region

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Family and Domains’ section describes a region of interest that cannot be described in other subsections.<p><a href='/help/region' target='_top'>More...</a></p>Regioni1121 – 1394RNA-bindingAdd BLAST274

Keywords - Domaini

Repeat, Transit peptide

Phylogenomic databases

evolutionary genealogy of genes: Non-supervised Orthologous Groups

More...
eggNOGi
KOG4318 Eukaryota
ENOG410XSG9 LUCA

Ensembl GeneTree

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GeneTreei
ENSGT00390000016775

The HOVERGEN Database of Homologous Vertebrate Genes

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HOVERGENi
HBG097314

InParanoid: Eukaryotic Ortholog Groups

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InParanoidi
P42704

KEGG Orthology (KO)

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KOi
K17964

Identification of Orthologs from Complete Genome Data

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OMAi
FFLRHCV

Database of Orthologous Groups

More...
OrthoDBi
EOG091G06CN

Database for complete collections of gene phylogenies

More...
PhylomeDBi
P42704

TreeFam database of animal gene trees

More...
TreeFami
TF323626

Family and domain databases

Gene3D Structural and Functional Annotation of Protein Families

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Gene3Di
1.25.40.10, 3 hits

Integrated resource of protein families, domains and functional sites

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InterProi
View protein in InterPro
IPR033490 LRP130
IPR002885 Pentatricopeptide_repeat
IPR033443 PPR_long
IPR011990 TPR-like_helical_dom_sf

The PANTHER Classification System

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PANTHERi
PTHR24015:SF945 PTHR24015:SF945, 2 hits

Pfam protein domain database

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Pfami
View protein in Pfam
PF01535 PPR, 3 hits
PF13812 PPR_3, 1 hit
PF17177 PPR_long, 1 hit

TIGRFAMs; a protein family database

More...
TIGRFAMsi
TIGR00756 PPR, 2 hits

PROSITE; a protein domain and family database

More...
PROSITEi
View protein in PROSITE
PS51375 PPR, 11 hits

<p>This section displays by default the canonical protein sequence and upon request all isoforms described in the entry. It also includes information pertinent to the sequence(s), including <a href="http://www.uniprot.org/help/sequence_length">length</a> and <a href="http://www.uniprot.org/help/sequences">molecular weight</a>.<p><a href='/help/sequences_section' target='_top'>More...</a></p>Sequence (1+)i

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is complete or not.<p><a href='/help/sequence_status' target='_top'>More...</a></p>Sequence statusi: Complete.

<p>This subsection of the <a href="http://www.uniprot.org/help/sequences_section">Sequence</a> section indicates if the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> displayed by default in the entry is in its mature form or if it represents the precursor.<p><a href='/help/sequence_processing' target='_top'>More...</a></p>Sequence processingi: The displayed sequence is further processed into a mature form.

This entry has 1 described isoform and 4 potential isoforms that are computationally mapped.Show allAlign All

P42704-1 [UniParc]FASTAAdd to basket
« Hide
        10         20         30         40         50
MAALLRSARW LLRAGAAPRL PLSLRLLPGG PGRLHAASYL PAARAGPVAG
60 70 80 90 100
GLLSPARLYA IAAKEKDIQE ESTFSSRKIS NQFDWALMRL DLSVRRTGRI
110 120 130 140 150
PKKLLQKVFN DTCRSGGLGG SHALLLLRSC GSLLPELKLE ERTEFAHRIW
160 170 180 190 200
DTLQKLGAVY DVSHYNALLK VYLQNEYKFS PTDFLAKMEE ANIQPNRVTY
210 220 230 240 250
QRLIASYCNV GDIEGASKIL GFMKTKDLPV TEAVFSALVT GHARAGDMEN
260 270 280 290 300
AENILTVMRD AGIEPGPDTY LALLNAYAEK GDIDHVKQTL EKVEKSELHL
310 320 330 340 350
MDRDLLQIIF SFSKAGYPQY VSEILEKVTC ERRYIPDAMN LILLLVTEKL
360 370 380 390 400
EDVALQILLA CPVSKEDGPS VFGSFFLQHC VTMNTPVEKL TDYCKKLKEV
410 420 430 440 450
QMHSFPLQFT LHCALLANKT DLAKALMKAV KEEGFPIRPH YFWPLLVGRR
460 470 480 490 500
KEKNVQGIIE ILKGMQELGV HPDQETYTDY VIPCFDSVNS ARAILQENGC
510 520 530 540 550
LSDSDMFSQA GLRSEAANGN LDFVLSFLKS NTLPISLQSI RSSLLLGFRR
560 570 580 590 600
SMNINLWSEI TELLYKDGRY CQEPRGPTEA VGYFLYNLID SMSDSEVQAK
610 620 630 640 650
EEHLRQYFHQ LEKMNVKIPE NIYRGIRNLL ESYHVPELIK DAHLLVESKN
660 670 680 690 700
LDFQKTVQLT SSELESTLET LKAENQPIRD VLKQLILVLC SEENMQKALE
710 720 730 740 750
LKAKYESDMV TGGYAALINL CCRHDKVEDA LNLKEEFDRL DSSAVLDTGK
760 770 780 790 800
YVGLVRVLAK HGKLQDAINI LKEMKEKDVL IKDTTALSFF HMLNGAALRG
810 820 830 840 850
EIETVKQLHE AIVTLGLAEP STNISFPLVT VHLEKGDLST ALEVAIDCYE
860 870 880 890 900
KYKVLPRIHD VLCKLVEKGE TDLIQKAMDF VSQEQGEMVM LYDLFFAFLQ
910 920 930 940 950
TGNYKEAKKI IETPGIRARS ARLQWFCDRC VANNQVETLE KLVELTQKLF
960 970 980 990 1000
ECDRDQMYYN LLKLYKINGD WQRADAVWNK IQEENVIPRE KTLRLLAEIL
1010 1020 1030 1040 1050
REGNQEVPFD VPELWYEDEK HSLNSSSAST TEPDFQKDIL IACRLNQKKG
1060 1070 1080 1090 1100
AYDIFLNAKE QNIVFNAETY SNLIKLLMSE DYFTQAMEVK AFAETHIKGF
1110 1120 1130 1140 1150
TLNDAANSRL IITQVRRDYL KEAVTTLKTV LDQQQTPSRL AVTRVIQALA
1160 1170 1180 1190 1200
MKGDVENIEV VQKMLNGLED SIGLSKMVFI NNIALAQIKN NNIDAAIENI
1210 1220 1230 1240 1250
ENMLTSENKV IEPQYFGLAY LFRKVIEEQL EPAVEKISIM AERLANQFAI
1260 1270 1280 1290 1300
YKPVTDFFLQ LVDAGKVDDA RALLQRCGAI AEQTPILLLF LLRNSRKQGK
1310 1320 1330 1340 1350
ASTVKSVLEL IPELNEKEEA YNSLMKSYVS EKDVTSAKAL YEHLTAKNTK
1360 1370 1380 1390
LDDLFLKRYA SLLKYAGEPV PFIEPPESFE FYAQQLRKLR ENSS
Length:1,394
Mass (Da):157,905
Last modified:July 24, 2007 - v3
<p>The checksum is a form of redundancy check that is calculated from the sequence. It is useful for tracking sequence updates.</p> <p>It should be noted that while, in theory, two different sequences could have the same checksum value, the likelihood that this would happen is extremely low.</p> <p>However UniProtKB may contain entries with identical sequences in case of multiple genes (paralogs).</p> <p>The checksum is computed as the sequence 64-bit Cyclic Redundancy Check value (CRC64) using the generator polynomial: x<sup>64</sup> + x<sup>4</sup> + x<sup>3</sup> + x + 1. The algorithm is described in the ISO 3309 standard. </p> <p class="publication">Press W.H., Flannery B.P., Teukolsky S.A. and Vetterling W.T.<br /> <strong>Cyclic redundancy and other checksums</strong><br /> <a href="http://www.nrbook.com/b/bookcpdf.php">Numerical recipes in C 2nd ed., pp896-902, Cambridge University Press (1993)</a>)</p> Checksum:i61AB0C8BF8A972E6
GO

<p>In eukaryotic reference proteomes, unreviewed entries that are likely to belong to the same gene are computationally mapped, based on gene identifiers from Ensembl, EnsemblGenomes and model organism databases.<p><a href='/help/gene_centric_isoform_mapping' target='_top'>More...</a></p>Computationally mapped potential isoform sequencesi

There are 4 potential isoforms mapped to this entry.BLASTAlignShow allAdd to basket
EntryEntry nameProtein names
Gene namesLengthAnnotation
B8ZZ38B8ZZ38_HUMAN
Leucine-rich PPR motif-containing p...
LRPPRC
504Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
C9JCA9C9JCA9_HUMAN
Leucine-rich PPR motif-containing p...
LRPPRC
359Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
A0A0C4DG06A0A0C4DG06_HUMAN
Leucine-rich PPR motif-containing p...
LRPPRC
531Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>
H7C3W8H7C3W8_HUMAN
Leucine-rich PPR motif-containing p...
LRPPRC
77Annotation score:

Annotation score:1 out of 5

<p>The annotation score provides a heuristic measure of the annotation content of a UniProtKB entry or proteome. This score <strong>cannot</strong> be used as a measure of the accuracy of the annotation as we cannot define the ‘correct annotation’ for any given protein.<p><a href='/help/annotation_score' target='_top'>More...</a></p>

<p>This subsection of the ‘Sequence’ section reports difference(s) between the protein sequence shown in the UniProtKB entry and other available protein sequences derived from the same gene.<p><a href='/help/sequence_caution' target='_top'>More...</a></p>Sequence cautioni

The sequence AAA67549 differs from that shown. Reason: Frameshift at several positions.Curated
The sequence AAA67549 differs from that shown. Reason: Erroneous initiation. Translation N-terminally extended.Curated

Experimental Info

Feature keyPosition(s)DescriptionActionsGraphical viewLength
<p>This subsection of the ‘Sequence’ section reports difference(s) between the canonical sequence (displayed by default in the entry) and the different sequence submissions merged in the entry. These various submissions may originate from different sequencing projects, different types of experiments, or different biological samples. Sequence conflicts are usually of unknown origin.<p><a href='/help/conflict' target='_top'>More...</a></p>Sequence conflicti54S → G in BAF82705 (PubMed:14702039).Curated1
Sequence conflicti296S → F in AAA67549 (PubMed:8012652).Curated1
Sequence conflicti528 – 531LKSN → YFPI in AAH26034 (PubMed:15489334).Curated4
Sequence conflicti556L → V in AAA67549 (PubMed:8012652).Curated1
Sequence conflicti583Y → N in AAA67549 (PubMed:8012652).Curated1
Sequence conflicti648S → R in AAA67549 (PubMed:8012652).Curated1
Sequence conflicti676Q → R in BAC86287 (PubMed:14702039).Curated1
Sequence conflicti702K → R in BAC86287 (PubMed:14702039).Curated1
Sequence conflicti750 – 752KYV → NYL in AAA67549 (PubMed:8012652).Curated3
Sequence conflicti769N → K in AAA67549 (PubMed:8012652).Curated1
Sequence conflicti1192N → D in BAC86287 (PubMed:14702039).Curated1

Natural variant

Feature keyPosition(s)DescriptionActionsGraphical viewLength
Natural variantiVAR_018656354A → V in LSFC. 1 PublicationCorresponds to variant dbSNP:rs119466000EnsemblClinVar.1
Natural variantiVAR_052935478T → A. Corresponds to variant dbSNP:rs35035668EnsemblClinVar.1
Natural variantiVAR_075428866Missing in LSFC; unknown pathological significance. 1 Publication1
Natural variantiVAR_075429909Missing in LSFC; unknown pathological significance. 1 Publication1

Sequence databases

Select the link destinations:

EMBL nucleotide sequence database

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EMBLi

GenBank nucleotide sequence database

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GenBanki

DNA Data Bank of Japan; a nucleotide sequence database

More...
DDBJi
Links Updated
AY289212 mRNA Translation: AAP41922.1
AK125781 mRNA Translation: BAC86287.1
AK290016 mRNA Translation: BAF82705.1
AC108476 Genomic DNA Translation: AAY24012.1
AC127379 Genomic DNA Translation: AAY24043.1
BC010282 mRNA Translation: AAH10282.1
BC026034 mRNA Translation: AAH26034.1
BC050311 mRNA Translation: AAH50311.1
BC130285 mRNA Translation: AAI30286.1
M92439 mRNA Translation: AAA67549.1 Sequence problems.

The Consensus CDS (CCDS) project

More...
CCDSi
CCDS33189.1

Protein sequence database of the Protein Information Resource

More...
PIRi
S27954

NCBI Reference Sequences

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RefSeqi
NP_573566.2, NM_133259.3

UniGene gene-oriented nucleotide sequence clusters

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UniGenei
Hs.368084

Genome annotation databases

Ensembl eukaryotic genome annotation project

More...
Ensembli
ENST00000260665; ENSP00000260665; ENSG00000138095

Database of genes from NCBI RefSeq genomes

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GeneIDi
10128

KEGG: Kyoto Encyclopedia of Genes and Genomes

More...
KEGGi
hsa:10128

UCSC genome browser

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UCSCi
uc002rtr.3 human

Keywords - Coding sequence diversityi

Polymorphism

<p>This section provides links to proteins that are similar to the protein sequence(s) described in this entry at different levels of sequence identity thresholds (100%, 90% and 50%) based on their membership in UniProt Reference Clusters (<a href="http://www.uniprot.org/help/uniref">UniRef</a>).<p><a href='/help/similar_proteins_section' target='_top'>More...</a></p>Similar proteinsi

<p>This section is used to point to information related to entries and found in data collections other than UniProtKB.<p><a href='/help/cross_references_section' target='_top'>More...</a></p>Cross-referencesi

Sequence databases

Select the link destinations:
EMBLi
GenBanki
DDBJi
Links Updated
AY289212 mRNA Translation: AAP41922.1
AK125781 mRNA Translation: BAC86287.1
AK290016 mRNA Translation: BAF82705.1
AC108476 Genomic DNA Translation: AAY24012.1
AC127379 Genomic DNA Translation: AAY24043.1
BC010282 mRNA Translation: AAH10282.1
BC026034 mRNA Translation: AAH26034.1
BC050311 mRNA Translation: AAH50311.1
BC130285 mRNA Translation: AAI30286.1
M92439 mRNA Translation: AAA67549.1 Sequence problems.
CCDSiCCDS33189.1
PIRiS27954
RefSeqiNP_573566.2, NM_133259.3
UniGeneiHs.368084

3D structure databases

ProteinModelPortaliP42704
ModBaseiSearch...
MobiDBiSearch...

Protein-protein interaction databases

BioGridi115432, 193 interactors
CORUMiP42704
DIPiDIP-27543N
IntActiP42704, 77 interactors
MINTiP42704
STRINGi9606.ENSP00000260665

PTM databases

CarbonylDBiP42704
iPTMnetiP42704
PhosphoSitePlusiP42704
SwissPalmiP42704

Polymorphism and mutation databases

BioMutaiLRPPRC
DMDMi156632706

Proteomic databases

EPDiP42704
MaxQBiP42704
PaxDbiP42704
PeptideAtlasiP42704
PRIDEiP42704
ProteomicsDBi55547

Protocols and materials databases

Structural Biology KnowledgebaseSearch...

Genome annotation databases

EnsembliENST00000260665; ENSP00000260665; ENSG00000138095
GeneIDi10128
KEGGihsa:10128
UCSCiuc002rtr.3 human

Organism-specific databases

Comparative Toxicogenomics Database

More...
CTDi
10128
DisGeNETi10128
EuPathDBiHostDB:ENSG00000138095.18

GeneCards: human genes, protein and diseases

More...
GeneCardsi
LRPPRC

H-Invitational Database, human transcriptome db

More...
H-InvDBi
HIX0023918
HGNCiHGNC:15714 LRPPRC
HPAiCAB033786
HPA036408
HPA036409
MalaCardsiLRPPRC
MIMi220111 phenotype
607544 gene
neXtProtiNX_P42704
OpenTargetsiENSG00000138095
Orphaneti70472 Congenital lactic acidosis, Saguenay-Lac-Saint-Jean type
PharmGKBiPA30459

GenAtlas: human gene database

More...
GenAtlasi
Search...

Phylogenomic databases

eggNOGiKOG4318 Eukaryota
ENOG410XSG9 LUCA
GeneTreeiENSGT00390000016775
HOVERGENiHBG097314
InParanoidiP42704
KOiK17964
OMAiFFLRHCV
OrthoDBiEOG091G06CN
PhylomeDBiP42704
TreeFamiTF323626

Enzyme and pathway databases

ReactomeiR-HSA-5628897 TP53 Regulates Metabolic Genes
R-HSA-611105 Respiratory electron transport

Miscellaneous databases

ChiTaRS: a database of human, mouse and fruit fly chimeric transcripts and RNA-sequencing data

More...
ChiTaRSi
LRPPRC human

The Gene Wiki collection of pages on human genes and proteins

More...
GeneWikii
LRPPRC

Database of phenotypes from RNA interference screens in Drosophila and Homo sapiens

More...
GenomeRNAii
10128

Protein Ontology

More...
PROi
PR:P42704

The Stanford Online Universal Resource for Clones and ESTs

More...
SOURCEi
Search...

Gene expression databases

BgeeiENSG00000138095 Expressed in 242 organ(s), highest expression level in skeletal muscle tissue of rectus abdominis
CleanExiHS_LRPPRC
ExpressionAtlasiP42704 baseline and differential
GenevisibleiP42704 HS

Family and domain databases

Gene3Di1.25.40.10, 3 hits
InterProiView protein in InterPro
IPR033490 LRP130
IPR002885 Pentatricopeptide_repeat
IPR033443 PPR_long
IPR011990 TPR-like_helical_dom_sf
PANTHERiPTHR24015:SF945 PTHR24015:SF945, 2 hits
PfamiView protein in Pfam
PF01535 PPR, 3 hits
PF13812 PPR_3, 1 hit
PF17177 PPR_long, 1 hit
TIGRFAMsiTIGR00756 PPR, 2 hits
PROSITEiView protein in PROSITE
PS51375 PPR, 11 hits

ProtoNet; Automatic hierarchical classification of proteins

More...
ProtoNeti
Search...

<p>This section provides general information on the entry.<p><a href='/help/entry_information_section' target='_top'>More...</a></p>Entry informationi

<p>This subsection of the ‘Entry information’ section provides a mnemonic identifier for a UniProtKB entry, but it is not a stable identifier. Each reviewed entry is assigned a unique entry name upon integration into UniProtKB/Swiss-Prot.<p><a href='/help/entry_name' target='_top'>More...</a></p>Entry nameiLPPRC_HUMAN
<p>This subsection of the ‘Entry information’ section provides one or more accession number(s). These are stable identifiers and should be used to cite UniProtKB entries. Upon integration into UniProtKB, each entry is assigned a unique accession number, which is called ‘Primary (citable) accession number’.<p><a href='/help/accession_numbers' target='_top'>More...</a></p>AccessioniPrimary (citable) accession number: P42704
Secondary accession number(s): A0PJE3
, A8K1V1, Q53PC0, Q53QN7, Q6ZUD8, Q7Z7A6, Q96D84
<p>This subsection of the ‘Entry information’ section shows the date of integration of the entry into UniProtKB, the date of the last sequence update and the date of the last annotation modification (‘Last modified’). The version number for both the entry and the <a href="http://www.uniprot.org/help/canonical_and_isoforms">canonical sequence</a> are also displayed.<p><a href='/help/entry_history' target='_top'>More...</a></p>Entry historyiIntegrated into UniProtKB/Swiss-Prot: November 1, 1995
Last sequence update: July 24, 2007
Last modified: December 5, 2018
This is version 181 of the entry and version 3 of the sequence. See complete history.
<p>This subsection of the ‘Entry information’ section indicates whether the entry has been manually annotated and reviewed by UniProtKB curators or not, in other words, if the entry belongs to the Swiss-Prot section of UniProtKB (<strong>reviewed</strong>) or to the computer-annotated TrEMBL section (<strong>unreviewed</strong>).<p><a href='/help/entry_status' target='_top'>More...</a></p>Entry statusiReviewed (UniProtKB/Swiss-Prot)
Annotation programChordata Protein Annotation Program
DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

<p>This section contains any relevant information that doesn’t fit in any other defined sections<p><a href='/help/miscellaneous_section' target='_top'>More...</a></p>Miscellaneousi

Keywords - Technical termi

Complete proteome, Direct protein sequencing, Reference proteome

Documents

  1. Human chromosome 2
    Human chromosome 2: entries, gene names and cross-references to MIM
  2. Human polymorphisms and disease mutations
    Index of human polymorphisms and disease mutations
  3. Human entries with polymorphisms or disease mutations
    List of human entries with polymorphisms or disease mutations
  4. MIM cross-references
    Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
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