Property Summary

NCBI Gene PubMed Count 45
Grant Count 13
R01 Count 10
Funding $2,264,008.9
PubMed Score 168.23
PubTator Score 215.49

Knowledge Summary

Patent

No data available

Expression

  Differential Expression (3)

Disease log2 FC p
psoriasis 3.100 0.000
diabetes mellitus -1.300 0.002
ovarian cancer 1.500 0.000

Gene RIF (29)

PMID Text
27033304 Mutations of Lon, which likely impair its chaperone properties, are at the basis of a genetic inherited disease named the cerebral, ocular, dental, auricular, skeletal (CODAS) syndrome. (Review)
26627475 This analysis revealed that LONM specifically recognises and degrades unfolded, but not aggregated proteins.
26363553 A review on the recent discoveries concerning Lon Protease functions. [review]
26314956 Inhibition of Lon protease by triterpenoids alters mitochondria and is associated to cell death in human cancer cells.
25922169 Lon downregulation attenuated hypoxia-induced cardiomyocyte apoptosis through a reduction of reactive oxygen species level.
25808063 LONP1 encodes an enzyme of bacterial ancestry that participates in protein turnover within the mitochondrial matrix, and mutations in its ATP-binding and proteolytic domains cause CODAS syndrome.
25675302 These results suggest that the mechanism underlying cell survival regulated by Lon is mediated by the maintenance of the protein stability of Hsp60-mtHsp70 complex.
25574826 We establish a link between LONP1 and CODAS syndrome in humans.
25369343 the structure of human mitochondrial Lon (hLon) protease, is reported.
25128872 Silencing of SIRT3 increased the levels of Lon protein and of its acetylation, suggesting that Lon is a target of SIRT3, likely at K917.
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AA Sequence

MAASTGYVRLWGAARCWVLRRPMLAAAGGRVPTAAGAWLLRGQRTCDASPPWALWGRGPAIGGQWRGFWE      1 - 70
ASSRGGGAFSGGEDASEGGAEEGAGGAGGSAGAGEGPVITALTPMTIPDVFPHLPLIAITRNPVFPRFIK     71 - 140
IIEVKNKKLVELLRRKVRLAQPYVGVFLKRDDSNESDVVESLDEIYHTGTFAQIHEMQDLGDKLRMIVMG    141 - 210
HRRVHISRQLEVEPEEPEAENKHKPRRKSKRGKKEAEDELSARHPAELAMEPTPELPAEVLMVEVENVVH    211 - 280
EDFQVTEEVKALTAEIVKTIRDIIALNPLYRESVLQMMQAGQRVVDNPIYLSDMGAALTGAESHELQDVL    281 - 350
EETNIPKRLYKALSLLKKEFELSKLQQRLGREVEEKIKQTHRKYLLQEQLKIIKKELGLEKDDKDAIEEK    351 - 420
FRERLKELVVPKHVMDVVDEELSKLGLLDNHSSEFNVTRNYLDWLTSIPWGKYSNENLDLARAQAVLEED    421 - 490
HYGMEDVKKRILEFIAVSQLRGSTQGKILCFYGPPGVGKTSIARSIARALNREYFRFSVGGMTDVAEIKG    491 - 560
HRRTYVGAMPGKIIQCLKKTKTENPLILIDEVDKIGRGYQGDPSSALLELLDPEQNANFLDHYLDVPVDL    561 - 630
SKVLFICTANVTDTIPEPLRDRMEMINVSGYVAQEKLAIAERYLVPQARALCGLDESKAKLSSDVLTLLI    631 - 700
KQYCRESGVRNLQKQVEKVLRKSAYKIVSGEAESVEVTPENLQDFVGKPVFTVERMYDVTPPGVVMGLAW    701 - 770
TAMGGSTLFVETSLRRPQDKDAKGDKDGSLEVTGQLGEVMKESARIAYTFARAFLMQHAPANDYLVTSHI    771 - 840
HLHVPEGATPKDGPSAGCTIVTALLSLAMGRPVRQNLAMTGEVSLTGKILPVGGIKEKTIAAKRAGVTCI    841 - 910
VLPAENKKDFYDLAAFITEGLEVHFVEHYREIFDIAFPDEQAEALAVER                         911 - 959
//

Text Mined References (53)

PMID Year Title
27033304 2016 Emerging role of Lon protease as a master regulator of mitochondrial functions.
26627475 2015 LON is the master protease that protects against protein aggregation in human mitochondria through direct degradation of misfolded proteins.
26363553 2015 Mitochondrial Lon protease at the crossroads of oxidative stress, ageing and cancer.
26314956 2015 Inhibition of Lon protease by triterpenoids alters mitochondria and is associated to cell death in human cancer cells.
25944712 2015 N-terminome analysis of the human mitochondrial proteome.
25922169 2015 Mitochondrial Lon protease controls ROS-dependent apoptosis in cardiomyocyte under hypoxia.
25808063 2015 Mutations in LONP1, a mitochondrial matrix protease, cause CODAS syndrome.
25675302 2015 Mitochondrial Lon regulates apoptosis through the association with Hsp60-mtHsp70 complex.
25574826 2015 CODAS syndrome is associated with mutations of LONP1, encoding mitochondrial AAA+ Lon protease.
25369343 2014 Three-dimensional reconstruction of the S885A mutant of human mitochondrial Lon protease.
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