EuRBPDB

Basic Information
Cancer Related Information

Description

RBPome

Literatures

Expression

Transcripts

Gene Model

Phenotypes

PPI

Orthologs

Gene Ontology

Description

Ensembl ID: ENSG00000196365 (Gene tree)
Gene ID: 9361
Gene Symbol: LONP1
Alias: PRSS15|LONP
Full Name: Lon Peptidase 1, Mitochondrial
Gene Type: Protein Coding
Species: Homo_sapiens
Status: confidence
Strand: Minus strand
Length: 28,739 bases
Position: chr19:5,691,834-5,720,572
Accession: 9479
RBP type: non-canonical RBP
Summary: This gene encodes a mitochondrial matrix protein that belongs to the Lon family of ATP-dependent proteases. This protein mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides in the mitochondrial matrix. It may also have a chaperone function in the assembly of inner membrane protein complexes, and participate in the regulation of mitochondrial gene expression and maintenance of the integrity of the mitochondrial genome. Decreased expression of this gene has been noted in a patient with hereditary spastic paraplegia (PMID:18378094). Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2013]

RNA binding proteome (RBPome)

PID	Title	Method	Time	Author	Doi
30352994	Discovery of RNA-binding proteins and characterization of their dynamic responses by enhanced RNA interactome capture	RIC & Jurkat	2018 Oct 23	Perez-Perri JI	DOI:10.1038/s41467-018-06557-8

Literatures on RNA binding capacity

PID	Title	Article	Time	Author	Doi
15090512	The protease Lon and the RNA-binding protein Hfq reduce silencing of the Escherichia coli bgl operon by H-NS.	J Bacteriol	2004 May	Dole S	-

Expression

Transcripts

Transcript ID	Name	Length	RefSeq ID	Protein ID	Length	RefSeq ID	UniportKB ID
ENST00000589473	LONP1-208	793	-	ENSP00000468379	264 (aa)	-	K7ERR6
ENST00000590206	LONP1-209	725	-	ENSP00000468083	242 (aa)	-	K7ER27
ENST00000590558	LONP1-211	2884	-	ENSP00000467808	185 (aa)	-	K7EQF8
ENST00000585374	LONP1-203	2882	-	ENSP00000465585	845 (aa)	-	K7EKE6
ENST00000588589	LONP1-207	684	-	-	- (aa)	-	-
ENST00000590728	LONP1-212	591	-	-	- (aa)	-	-
ENST00000360614	LONP1-201	3236	-	ENSP00000353826	959 (aa)	-	P36776
ENST00000590511	LONP1-210	722	-	-	- (aa)	-	-
ENST00000586617	LONP1-204	471	-	ENSP00000468385	86 (aa)	-	K7ERS1
ENST00000587365	LONP1-205	641	-	ENSP00000468114	210 (aa)	-	K7ER56
ENST00000591321	LONP1-214	913	-	-	- (aa)	-	-
ENST00000590729	LONP1-213	2626	-	ENSP00000465139	829 (aa)	-	K7EJE8
ENST00000540670	LONP1-202	3002	-	ENSP00000441523	763 (aa)	-	P36776
ENST00000587552	LONP1-206	2816	-	-	- (aa)	-	-
ENST00000593119	LONP1-215	2918	-	ENSP00000468541	895 (aa)	-	P36776

Gene Model

Click here to download ENSG00000196365's gene model file

Phenotypes

ensgID	Trait	pValue	Pubmed ID
ENSG00000196365	Platelet Function Tests	2.4475026E-006	-

Protein-Protein Interaction (PPI)

Clik here to download ENSG00000196365's network

* RBP PPI network refers to all genes directly bind to RBP

Orthologs identified by RBPome

Ensembl ID	Gene Symbol	Coverage	Identiy	Ortholog	Gene Symbol	Coverage	Identiy	Species
ENSG00000196365	LONP1	99	69.517	FBgn0036892	Lon	88	63.229	Drosophila_melanogaster
ENSG00000196365	LONP1	100	94.030	ENSMUSG00000041168	Lonp1	100	86.100	Mus_musculus

Gene Ontology

Go ID	Go_term	PubmedID	Evidence	Category
GO:0000002	mitochondrial genome maintenance	17420247.	NAS	Process
GO:0001018	mitochondrial promoter sequence-specific DNA binding	9485316.	IDA	Function
GO:0001018	mitochondrial promoter sequence-specific DNA binding	9485316.	IDA	Function
GO:0001666	response to hypoxia	17418790.	IEP	Process
GO:0003697	single-stranded DNA binding	21873635.	IBA	Function
GO:0003727	single-stranded RNA binding	14739292.	IDA	Function
GO:0004176	ATP-dependent peptidase activity	21873635.	IBA	Function
GO:0004176	ATP-dependent peptidase activity	8248235.17420247.	IDA	Function
GO:0004252	serine-type endopeptidase activity	-	IEA	Function
GO:0005515	protein binding	14739292.24520911.	IPI	Function
GO:0005524	ATP binding	14739292.	IDA	Function
GO:0005634	nucleus	-	IDA	Component
GO:0005654	nucleoplasm	-	IDA	Component
GO:0005739	mitochondrion	8248235.	IDA	Component
GO:0005759	mitochondrial matrix	21873635.	IBA	Component
GO:0005759	mitochondrial matrix	12198491.	IMP	Component
GO:0005829	cytosol	-	IDA	Component
GO:0006515	protein quality control for misfolded or incompletely synthesized proteins	21873635.	IBA	Process
GO:0007005	mitochondrion organization	21873635.	IBA	Process
GO:0007005	mitochondrion organization	15683722.	IMP	Process
GO:0007568	aging	-	IEA	Process
GO:0009725	response to hormone	-	IEA	Process
GO:0010044	response to aluminum ion	-	IEA	Process
GO:0016020	membrane	19946888.	HDA	Component
GO:0032042	mitochondrial DNA metabolic process	14739292.	NAS	Process
GO:0034599	cellular response to oxidative stress	12198491.	IC	Process
GO:0034599	cellular response to oxidative stress	17420247.	IDA	Process
GO:0042645	mitochondrial nucleoid	18063578.	IDA	Component
GO:0043531	ADP binding	14739292.	IDA	Function
GO:0043565	sequence-specific DNA binding	14739292.	IDA	Function
GO:0051131	chaperone-mediated protein complex assembly	21873635.	IBA	Process
GO:0051260	protein homooligomerization	14739292.	IDA	Process
GO:0051603	proteolysis involved in cellular protein catabolic process	8248235.17420247.	IDA	Process
GO:0051880	G-quadruplex DNA binding	18174225.	IDA	Function
GO:0070182	DNA polymerase binding	14739292.	IPI	Function
GO:0070407	oxidation-dependent protein catabolic process	12198491.	IMP	Process

Cancer associated literatures

PID	Title	Article	Time	Author	Doi
23788038	Overexpression of Lon contributes to survival and aggressive phenotype of cancer cells through mitochondrial complex I-mediated generation of reactive oxygen species.	Cell Death Dis	2013 Jun 20	Cheng CW	doi: 10.1038/cddis.2013.204.
26314956	Inhibition of Lon protease by triterpenoids alters mitochondria and is associated to cell death in human cancer cells.	Oncotarget	2015 Sep 22	Gibellini L	doi: 10.18632/oncotarget.4510.
24260536	Down-regulating overexpressed human Lon in cervical cancer suppresses cell proliferation and bioenergetics.	PLoS One	2013 Nov 19	Nie X	doi: 10.1371/journal.pone.0081084
29178076	Mitochondrial Lon Protease and Cancer.	Adv Exp Med Biol	2017	Lu B	doi: 10.1007/978-981-10-6674-0_12.

Differential Expression

Expression in 33 cancers

Mutations

Cancer	Chr	Position	Mutation Type	dbSNP	Protein-change	Allele Freq
ACC	chr19	5707744	Missense_Mutation	novel	T339P	0.16
BLCA	chr19	5699043	Missense_Mutation	rs752139036	E557K	0.17
BLCA	chr19	5707763	Silent	rs752556297	S332S	0.29
BLCA	chr19	5696162	Missense_Mutation	rs746046624	F635L	0.28
BLCA	chr19	5719782	Silent	rs746852002	I117I	0.33
BLCA	chr19	5696698	Missense_Mutation	rs140317309	T582M	0.73
BLCA	chr19	5714229	Missense_Mutation	rs370084099	R158C	0.44
BLCA	chr19	5692047	Silent	novel	L955L	0.21
BLCA	chr19	5693367	Silent	rs748466230	A878A	0.86
BLCA	chr19	5693368	Missense_Mutation	novel	A878G	0.86
BLCA	chr19	5711902	Missense_Mutation	novel	E247K	0.21
BLCA	chr19	5711925	Missense_Mutation	NA	S239L	0.32
BLCA	chr19	5711800	Missense_Mutation	novel	E281Q	0.14
BLCA	chr19	5691990	3'UTR	novel		0.47
BLCA	chr19	5696300	Missense_Mutation	novel	Q615H	0.18
BRCA	chr19	5692061	Nonsense_Mutation	NA	Q951*	0.31
BRCA	chr19	5708341	Splice_Site	NA	X311_splice	0.67
CESC	chr19	5700804	Silent	rs561481182	V497V	0.14
CESC	chr19	5696105	Missense_Mutation	NA	E654D	0.13
CESC	chr19	5700852	Silent	rs146759309	A481A	0.34
COAD	chr19	5693552	Splice_Region	novel	E846E	0.29
COAD	chr19	5693733	Missense_Mutation	rs756015513	R786Q	0.26
COAD	chr19	5707821	Missense_Mutation	novel	S313L	0.35
COAD	chr19	5719797	Silent	novel	L112L	0.21
COAD	chr19	5714206	Silent	rs146460691	G165G	0.36
COAD	chr19	5694887	Silent	NA	P676P	0.15
COAD	chr19	5696142	Missense_Mutation	rs371606894	T642M	0.42
COAD	chr19	5707714	Missense_Mutation	rs199733142	V349I	0.22
COAD	chr19	5719966	Missense_Mutation	novel	G56V	0.1
COAD	chr19	5707131	Silent	novel	L359L	0.35
COAD	chr19	5692156	Missense_Mutation	novel	D919V	0.23
COAD	chr19	5696337	Missense_Mutation	rs746766577	P603L	0.27
COAD	chr19	5692082	Missense_Mutation	rs138631909	D944N	0.15
COAD	chr19	5712043	Intron	novel		0.61
COAD	chr19	5694879	Missense_Mutation	rs549574673	R679H	0.33
COAD	chr19	5696258	Silent	NA	D629D	0.5
COAD	chr19	5694833	Silent	rs770545248	D694D	0.29
COAD	chr19	5696755	Missense_Mutation	novel	R563Q	0.11
ESCA	chr19	5692035	Silent	rs150782036	R959R	0.72
ESCA	chr19	5713245	Missense_Mutation	rs139555567	S176L	0.43
ESCA	chr19	5694516	Missense_Mutation	rs768949488	E731K	0.42
GBM	chr19	5700829	Missense_Mutation	novel	E489A	0.04
GBM	chr19	5714193	Silent	novel	R170R	0.16
GBM	chr19	5700839	Missense_Mutation	novel	V486L	0.12
GBM	chr19	5713229	Missense_Mutation	novel	S181R	0.12
GBM	chr19	5713211	Missense_Mutation	novel	H187Q	0.1
GBM	chr19	5694539	Missense_Mutation	rs763812313	S723L	0.15
GBM	chr19	5694878	Silent	rs151150480	R679R	0.25
GBM	chr19	5713235	Silent	rs772056590	V179V	0.48
HNSC	chr19	5711874	Missense_Mutation	rs116591583	A256V	0.25
HNSC	chr19	5693336	In_Frame_Del	novel	K888del	0.33
HNSC	chr19	5699104	Silent	rs376139384	I536I	0.18
HNSC	chr19	5700877	Missense_Mutation	novel	Y473C	0.25
HNSC	chr19	5692077	Frame_Shift_Ins	novel	*A946Sfs71**	0.2
HNSC	chr19	5719894	Missense_Mutation	rs781065201	S80L	0.2
KICH	chr19	5707735	Missense_Mutation	rs748543089	E342K	0.31
KIRC	chr19	5699076	Missense_Mutation	NA	R546G	0.23
KIRC	chr19	5693645	Silent	novel	R815R	0.29
LGG	chr19	5692211	Splice_Region	novel		0.08
LGG	chr19	5691988	3'UTR	novel		0.03
LIHC	chr19	5699200	Silent	rs763432896	F504F	0.63
LIHC	chr19	5719812	Silent	rs760045514	P107P	0.14
LUAD	chr19	5720011	Missense_Mutation	NA	R41Q	0.6
LUAD	chr19	5700833	Missense_Mutation	novel	E488Q	0.13
LUAD	chr19	5711887	Missense_Mutation	novel	A252S	0.46
LUAD	chr19	5711888	Missense_Mutation	novel	S251R	0.47
LUAD	chr19	5705930	Silent	rs764862589	I403I	0.22
LUAD	chr19	5705915	Silent	novel	G408G	0.22
LUAD	chr19	5699059	Frame_Shift_Ins	novel	*M552Hfs3**	0.14
LUAD	chr19	5711994	Missense_Mutation	NA	I216S	0.29
LUAD	chr19	5711925	Nonsense_Mutation	novel	S239*	0.19
LUSC	chr19	5705947	Nonsense_Mutation	novel	E398*	0.4
LUSC	chr19	5693536	Splice_Site	novel	X846_splice	0.09
LUSC	chr19	5693444	Missense_Mutation	novel	G853C	0.18
LUSC	chr19	5696750	Missense_Mutation	rs764931850	Y565H	0.19
LUSC	chr19	5711815	Missense_Mutation	NA	E276Q	0.06
OV	chr19	5707736	Silent	rs146893125	A341A	0.19
OV	chr19	5711903	Silent	NA	A246A	0.26
OV	chr19	5713203	Missense_Mutation	rs748610478	T190M	0.31
OV	chr19	5700862	Missense_Mutation	novel	L478Q	0.31
PAAD	chr19	5693647	Missense_Mutation	rs748370005	R815C	0.06
PAAD	chr19	5693641	Missense_Mutation	novel	A817S	0.23
PAAD	chr19	5694900	Missense_Mutation	rs138134205	R672H	0.15
PAAD	chr19	5714223	Missense_Mutation	rs148374055	A160T	0.2
PCPG	chr19	5692043	Missense_Mutation	novel	V957L	0.46
PRAD	chr19	5692201	Frame_Shift_Ins	novel	*R904Lfs5**	0.05
PRAD	chr19	5711832	Missense_Mutation	novel	V270G	0.09
PRAD	chr19	5707715	Silent	rs751274908	D348D	0.43
READ	chr19	5693594	Silent	novel	N832N	0.09
READ	chr19	5700796	Missense_Mutation	rs368654972	R500H	0.16
SARC	chr19	5707136	Missense_Mutation	novel	K357T	0.28
SARC	chr19	5691996	3'UTR	novel		0.02
SARC	chr19	5694794	Silent	rs376954188	S707S	0.43
SKCM	chr19	5714211	Missense_Mutation	NA	V164F	0.32
SKCM	chr19	5705914	Silent	NA	L409L	0.16
SKCM	chr19	5713189	Missense_Mutation	rs770702880	H195Y	0.36
SKCM	chr19	5707114	Silent	novel	S364S	0.45
SKCM	chr19	5711841	Missense_Mutation	rs759923141	P267L	0.15
STAD	chr19	5705836	Missense_Mutation	NA	M435V	0.32
STAD	chr19	5694781	Silent	NA	L712L	0.37
STAD	chr19	5699099	Missense_Mutation	rs115968490	R538H	0.32
STAD	chr19	5699193	Missense_Mutation	rs143983421	V507I	0.42
STAD	chr19	5711810	Silent	rs776204640	N277N	0.22
STAD	chr19	5713209	Missense_Mutation	rs147052599	T188M	0.34
STAD	chr19	5693396	Frame_Shift_Ins	novel	*M869Pfs12**	0.27
STAD	chr19	5692111	Missense_Mutation	NA	H934R	0.2
STAD	chr19	5696682	Silent	novel	I587I	0.29
STAD	chr19	5705897	Missense_Mutation	novel	K414N	0.42
STAD	chr19	5708372	Missense_Mutation	novel	R301Q	0.23
STAD	chr19	5714182	Splice_Site	NA	X173_splice	0.28
STAD	chr19	5693661	Missense_Mutation	NA	M810T	0.27
STAD	chr19	5700852	Silent	rs146759309	A481A	0.28
STAD	chr19	5692148	Missense_Mutation	rs139476430	D922N	0.75
STAD	chr19	5700797	Missense_Mutation	rs377243139	R500C	0.46
STAD	chr19	5693343	Silent	rs142397293	T886T	0.52
STAD	chr19	5700813	Missense_Mutation	NA	M494I	0.08
STAD	chr19	5699200	Silent	rs763432896	F504F	0.3
TGCT	chr19	5693398	Missense_Mutation	novel	A868V	0.07
TGCT	chr19	5696668	Splice_Site	novel	X588_splice	0.38
TGCT	chr19	5720003	Silent	novel	R44R	0.09
THCA	chr19	5691982	3'UTR	novel		0.05
THCA	chr19	5719894	Missense_Mutation	rs781065201	S80L	0.31
UCEC	chr19	5713151	Silent	rs765125145	I207I	0.18
UCEC	chr19	5699064	Missense_Mutation	novel	G550R	0.22
UCEC	chr19	5693343	Silent	rs142397293	T886T	0.48
UCEC	chr19	5699075	Missense_Mutation	rs148761187	R546H	0.38
UCEC	chr19	5714260	Missense_Mutation	NA	K147N	0.41
UCEC	chr19	5693415	Silent	novel	T862T	0.35
UCEC	chr19	5700889	Missense_Mutation	novel	P469H	0.43
UCEC	chr19	5699093	Missense_Mutation	NA	L540R	0.5
UCEC	chr19	5714229	Missense_Mutation	rs370084099	R158C	0.61
UCEC	chr19	5705876	Missense_Mutation	novel	F421L	0.07
UCEC	chr19	5714229	Missense_Mutation	rs370084099	R158C	0.2
UCEC	chr19	5711800	Missense_Mutation	NA	E281K	0.19
UCEC	chr19	5711898	Missense_Mutation	NA	D248G	0.19
UCEC	chr19	5694877	Missense_Mutation	rs754884886	A680T	0.18
UCEC	chr19	5700885	Nonsense_Mutation	novel	W470*	0.12
UCEC	chr19	5707696	Splice_Site	NA	X354_splice	0.64
UCEC	chr19	5711813	Silent	NA	E276E	0.55
UCEC	chr19	5700897	Silent	rs772469839	T466T	0.35
UCEC	chr19	5705861	Missense_Mutation	novel	K426N	0.34
UCEC	chr19	5711954	Silent	rs143270482	A229A	0.11
UCEC	chr19	5696687	Silent	novel	L586L	0.24
UCEC	chr19	5713244	Silent	rs147695228	S176S	0.15
UCEC	chr19	5700832	Missense_Mutation	novel	E488G	0.33
UCEC	chr19	5711934	Missense_Mutation	rs200781774	R236H	0.52
UCEC	chr19	5713202	Silent	rs781684306	T190T	0.35
UCEC	chr19	5693651	Silent	rs368187848	S813S	0.45
UCEC	chr19	5693343	Silent	rs142397293	T886T	0.22
UCEC	chr19	5696096	Silent	rs750236561	N657N	0.28
UCEC	chr19	5714208	Missense_Mutation	rs780360153	G165S	0.54
UCEC	chr19	5700897	Silent	rs772469839	T466T	0.31
UCEC	chr19	5713137	Missense_Mutation	NA	R212I	0.43
UCEC	chr19	5699138	Frame_Shift_Del	NA	*P525Lfs16**	0.29
UCEC	chr19	5699195	Missense_Mutation	novel	A506V	0.16
UCEC	chr19	5707122	Missense_Mutation	novel	A362S	0.18
UCEC	chr19	5707708	Missense_Mutation	rs57175043	E351K	0.09
UCEC	chr19	5693298	Splice_Region	rs527366898	A901A	0.3
UCEC	chr19	5693599	Missense_Mutation	rs141916070	A831T	0.35
UCEC	chr19	5696072	Silent	novel	E665E	0.24
UCEC	chr19	5699138	Frame_Shift_Del	NA	*P525Lfs16**	0.31
UCEC	chr19	5711903	Silent	NA	A246A	0.51
UCEC	chr19	5693399	Missense_Mutation	novel	A868T	0.24
UCEC	chr19	5711800	Missense_Mutation	NA	E281K	0.27
UCEC	chr19	5714265	Missense_Mutation	novel	N146H	0.19
UCEC	chr19	5691997	3'UTR	novel		0.22
UCEC	chr19	5693650	Missense_Mutation	rs566420786	A814T	0.17
UCEC	chr19	5707748	Silent	rs149402772	A337A	0.26
UCEC	chr19	5694795	Missense_Mutation	novel	S707N	0.49
UCEC	chr19	5711934	Missense_Mutation	rs200781774	R236H	0.4
UCEC	chr19	5694866	Silent	novel	G683G	0.24
UCEC	chr19	5714229	Missense_Mutation	rs370084099	R158C	0.36
UCEC	chr19	5705869	Missense_Mutation	novel	R424C	0.16
UCEC	chr19	5708403	Missense_Mutation	novel	A291T	0.38
UCEC	chr19	5691925	3'UTR	novel		0.44
UCEC	chr19	5693299	Missense_Mutation	rs562878082	A901V	0.34
UCEC	chr19	5696755	Missense_Mutation	novel	R563Q	0.47
UCEC	chr19	5700860	Missense_Mutation	novel	D479N	0.3

Copy Number Variations (CNVs)

Cancer	Type	Freq	Q-value
PAAD	DEL	0.1413	0.0060878
READ	DEL	0.1152	0.23023
THCA	DEL	0.012	0.10598

Survival Analysis

Cancer	P-value	Q-value
KIRC	0.041	Kaplan-Meier Survival Analysis
STAD	0.012	Kaplan-Meier Survival Analysis
MESO	0.016	Kaplan-Meier Survival Analysis
SKCM	0.00052	Kaplan-Meier Survival Analysis
LUSC	0.013	Kaplan-Meier Survival Analysis
PAAD	0.0033	Kaplan-Meier Survival Analysis
LIHC	0.007	Kaplan-Meier Survival Analysis
DLBC	0.043	Kaplan-Meier Survival Analysis

Drugs

EuRBPDB

Description

RBPome

Literatures

Expression

Transcripts

Gene Model

Phenotypes

PPI

Orthologs

Gene Ontology

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Eesembl ID

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