Property Summary

NCBI Gene PubMed Count 51
Grant Count 287
R01 Count 185
Funding $23,047,092.17
PubMed Score 48.17
PubTator Score 41.75

Knowledge Summary

Patent

No data available

Expression

Synonym

Accession P42704 A0PJE3 A8K1V1 Q53PC0 Q53QN7 Q6ZUD8 Q7Z7A6 Q96D84
Symbols LSFC
GP130
LRP130
CLONE-23970

Gene

 GWAS Trait (1)

Gene RIF (33)

PMID Text
26510951 study identifies LRPPRC as an important disease-causing gene in an early-onset, multisystem and neurological mitochondrial disease.
26412102 LRPPRC knock-down in mammalian cells leads to an imbalance between mitochondria-encoded and nuclear-encoded subunits of complex IV
25631043 Tetherin binds with the mitochondrion-associated autophagy suppressor LRPPRC and prohibits its association with the autophagy initiation complex.
25379610 Downregulation of LRPPRC expression resulted in the reduced expression of Bcl-2, upregulation of Bax, and cleaved caspase-9 and caspase-3. This induces apoptosis through the mitochondria-mediated pathway in PCa cells.
25214534 LRPPRC levels were reduced in muscle cells and undetectable in liver from French Canadian Leigh Syndrome patients.
25089713 LRPPRC is a transcription factor related to ABCB1 expression and highlight the importance of epigenetic regulation in CML resistance.
24722279 LRPPRC functions as a checkpoint protein that prevents mitochondria from autophagy degradation and impact tumorigenesis.
24375316 LRPPRC overexpression is associated with gastric cancer.
23878239 Data indicate that C14C10.4/MMA-1 Is the Structural and functional homolog of mammalian LRPPRC.
23822101 LRPPRC therefore acts to suppress the initiation of basal levels of autophagy to clean up dysfunctional mitochondria and other cellular debris during the normal cell cycle.
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AA Sequence

MAALLRSARWLLRAGAAPRLPLSLRLLPGGPGRLHAASYLPAARAGPVAGGLLSPARLYAIAAKEKDIQE      1 - 70
ESTFSSRKISNQFDWALMRLDLSVRRTGRIPKKLLQKVFNDTCRSGGLGGSHALLLLRSCGSLLPELKLE     71 - 140
ERTEFAHRIWDTLQKLGAVYDVSHYNALLKVYLQNEYKFSPTDFLAKMEEANIQPNRVTYQRLIASYCNV    141 - 210
GDIEGASKILGFMKTKDLPVTEAVFSALVTGHARAGDMENAENILTVMRDAGIEPGPDTYLALLNAYAEK    211 - 280
GDIDHVKQTLEKVEKSELHLMDRDLLQIIFSFSKAGYPQYVSEILEKVTCERRYIPDAMNLILLLVTEKL    281 - 350
EDVALQILLACPVSKEDGPSVFGSFFLQHCVTMNTPVEKLTDYCKKLKEVQMHSFPLQFTLHCALLANKT    351 - 420
DLAKALMKAVKEEGFPIRPHYFWPLLVGRRKEKNVQGIIEILKGMQELGVHPDQETYTDYVIPCFDSVNS    421 - 490
ARAILQENGCLSDSDMFSQAGLRSEAANGNLDFVLSFLKSNTLPISLQSIRSSLLLGFRRSMNINLWSEI    491 - 560
TELLYKDGRYCQEPRGPTEAVGYFLYNLIDSMSDSEVQAKEEHLRQYFHQLEKMNVKIPENIYRGIRNLL    561 - 630
ESYHVPELIKDAHLLVESKNLDFQKTVQLTSSELESTLETLKAENQPIRDVLKQLILVLCSEENMQKALE    631 - 700
LKAKYESDMVTGGYAALINLCCRHDKVEDALNLKEEFDRLDSSAVLDTGKYVGLVRVLAKHGKLQDAINI    701 - 770
LKEMKEKDVLIKDTTALSFFHMLNGAALRGEIETVKQLHEAIVTLGLAEPSTNISFPLVTVHLEKGDLST    771 - 840
ALEVAIDCYEKYKVLPRIHDVLCKLVEKGETDLIQKAMDFVSQEQGEMVMLYDLFFAFLQTGNYKEAKKI    841 - 910
IETPGIRARSARLQWFCDRCVANNQVETLEKLVELTQKLFECDRDQMYYNLLKLYKINGDWQRADAVWNK    911 - 980
IQEENVIPREKTLRLLAEILREGNQEVPFDVPELWYEDEKHSLNSSSASTTEPDFQKDILIACRLNQKKG    981 - 1050
AYDIFLNAKEQNIVFNAETYSNLIKLLMSEDYFTQAMEVKAFAETHIKGFTLNDAANSRLIITQVRRDYL   1051 - 1120
KEAVTTLKTVLDQQQTPSRLAVTRVIQALAMKGDVENIEVVQKMLNGLEDSIGLSKMVFINNIALAQIKN   1121 - 1190
NNIDAAIENIENMLTSENKVIEPQYFGLAYLFRKVIEEQLEPAVEKISIMAERLANQFAIYKPVTDFFLQ   1191 - 1260
LVDAGKVDDARALLQRCGAIAEQTPILLLFLLRNSRKQGKASTVKSVLELIPELNEKEEAYNSLMKSYVS   1261 - 1330
EKDVTSAKALYEHLTAKNTKLDDLFLKRYASLLKYAGEPVPFIEPPESFEFYAQQLRKLRENSS         1331 - 1394
//

Text Mined References (61)

PMID Year Title
26510951 2015 LRPPRC mutations cause early-onset multisystem mitochondrial disease outside of the French-Canadian population.
26496610 2015 A human interactome in three quantitative dimensions organized by stoichiometries and abundances.
26412102 2015 The loss of LRPPRC function induces the mitochondrial unfolded protein response.
25944712 2015 N-terminome analysis of the human mitochondrial proteome.
25631043 2015 The viral restriction factor tetherin prevents leucine-rich pentatricopeptide repeat-containing protein (LRPPRC) from association with beclin 1 and B-cell CLL/lymphoma 2 (Bcl-2) and enhances autophagy and mitophagy.
25379610 2014 Downregulation of LRPPRC induces apoptosis in prostate cancer cells through the mitochondria-mediated pathway.
25214534 2015 Tissue-specific responses to the LRPPRC founder mutation in French Canadian Leigh Syndrome.
25089713 2014 ABCB1 regulation through LRPPRC is influenced by the methylation status of the GC -100 box in its promoter.
24722279 2014 Autophagy inhibitor LRPPRC suppresses mitophagy through interaction with mitophagy initiator Parkin.
24375316 2014 The significance of LRPPRC overexpression in gastric cancer.
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