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Center for Computational Systems Medicine
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Fusion Gene Summary

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Fusion Gene ORF analysis

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Fusion Genomic Features

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Fusion Protein Features

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Fusion Gene Sequence

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Fusion Gene PPI analysis

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Related Drugs

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Related Diseases

Fusion gene:LONP1-DUS3L (FusionGDB2 ID:49414)

Fusion Gene Summary for LONP1-DUS3L

check button Fusion gene summary
Fusion gene informationFusion gene name: LONP1-DUS3L
Fusion gene ID: 49414
HgeneTgene
Gene symbol

LONP1

DUS3L

Gene ID

9361

56931

Gene namelon peptidase 1, mitochondrialdihydrouridine synthase 3 like
SynonymsCODASS|LON|LONP|LonHS|PIM1|PRSS15|hLONDUS3
Cytomap

19p13.3

19p13.3

Type of geneprotein-codingprotein-coding
Descriptionlon protease homolog, mitochondrialhLON ATP-dependent proteaselon protease-like proteinmitochondrial ATP-dependent protease Lonmitochondrial lon protease-like proteinserine protease 15tRNA-dihydrouridine(47) synthase [NAD(P)(+)]-like
Modification date2020031320200313
UniProtAcc

P36776

Q96G46

Ensembl transtripts involved in fusion geneENST00000360614, ENST00000540670, 
ENST00000585374, ENST00000590729, 
ENST00000593119, ENST00000590511, 
ENST00000590681, ENST00000309061, 
ENST00000320699, 
Fusion gene scores* DoF score18 X 14 X 12=30243 X 3 X 3=27
# samples 234
** MAII scorelog2(23/3024*10)=-3.7167523732767
possibly effective Gene in Pan-Cancer Fusion Genes (peGinPCFGs).
DoF>8 and MAII<0		log2(4/27*10)=0.567040592723894
effective Gene in Pan-Cancer Fusion Genes (eGinPCFGs).
DoF>8 and MAII>0
Context

PubMed: LONP1 [Title/Abstract] AND DUS3L [Title/Abstract] AND fusion [Title/Abstract]

Most frequent breakpointLONP1(5705782)-DUS3L(5790346), # samples:1
LONP1(5705783)-DUS3L(5790346), # samples:1
Anticipated loss of major functional domain due to fusion event.LONP1-DUS3L seems lost the major protein functional domain in Hgene partner, which is a essential gene due to the frame-shifted ORF.
* DoF score (Degree of Frequency) = # partners X # break points X # cancer types
** MAII score (Major Active Isofusion Index) = log2(# samples/DoF score*10)

check button Gene ontology of each fusion partner gene with evidence of Inferred from Direct Assay (IDA) from Entrez
PartnerGeneGO IDGO termPubMed ID
HgeneLONP1

GO:0034599

cellular response to oxidative stress

17420247

HgeneLONP1

GO:0051603

proteolysis involved in cellular protein catabolic process

8248235|17420247


check buttonFusion gene breakpoints across LONP1 (5'-gene)
* Click on the image to open the UCSC genome browser with custom track showing this image in a new window.
all structure

check buttonFusion gene breakpoints across DUS3L (3'-gene)
* Click on the image to open the UCSC genome browser with custom track showing this image in a new window.
all structure

check button Fusion gene information from two resources (ChiTars 5.0 and ChimerDB 4.0)
* All genome coordinats were lifted-over on hg19.
* Click on the break point to see the gene structure around the break point region using the UCSC Genome Browser.
SourceDiseaseSampleHgeneHchrHbpHstrandTgeneTchrTbpTstrand
ChimerDB4STADTCGA-BR-8289-01ALONP1chr19

5705783

-DUS3Lchr19

5790346

-
ChimerDB4STADTCGA-BR-8289LONP1chr19

5705782

-DUS3Lchr19

5790346

-


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Fusion Gene ORF analysis for LONP1-DUS3L

check button Open reading frame (ORF) analsis of fusion genes based on Ensembl gene isoform structure.
* Click on the break point to see the gene structure around the break point region using the UCSC Genome Browser.
ORFHenstTenstHgeneHchrHbpHstrandTgeneTchrTbpTstrand
5CDS-5UTRENST00000360614ENST00000590681LONP1chr19

5705783

-DUS3Lchr19

5790346

-
5CDS-5UTRENST00000360614ENST00000590681LONP1chr19

5705782

-DUS3Lchr19

5790346

-
5CDS-5UTRENST00000540670ENST00000590681LONP1chr19

5705783

-DUS3Lchr19

5790346

-
5CDS-5UTRENST00000540670ENST00000590681LONP1chr19

5705782

-DUS3Lchr19

5790346

-
5CDS-5UTRENST00000585374ENST00000590681LONP1chr19

5705783

-DUS3Lchr19

5790346

-
5CDS-5UTRENST00000585374ENST00000590681LONP1chr19

5705782

-DUS3Lchr19

5790346

-
5CDS-5UTRENST00000590729ENST00000590681LONP1chr19

5705783

-DUS3Lchr19

5790346

-
5CDS-5UTRENST00000590729ENST00000590681LONP1chr19

5705782

-DUS3Lchr19

5790346

-
5CDS-5UTRENST00000593119ENST00000590681LONP1chr19

5705783

-DUS3Lchr19

5790346

-
5CDS-5UTRENST00000593119ENST00000590681LONP1chr19

5705782

-DUS3Lchr19

5790346

-
Frame-shiftENST00000585374ENST00000309061LONP1chr19

5705783

-DUS3Lchr19

5790346

-
Frame-shiftENST00000585374ENST00000309061LONP1chr19

5705782

-DUS3Lchr19

5790346

-
Frame-shiftENST00000585374ENST00000320699LONP1chr19

5705783

-DUS3Lchr19

5790346

-
Frame-shiftENST00000585374ENST00000320699LONP1chr19

5705782

-DUS3Lchr19

5790346

-
In-frameENST00000360614ENST00000309061LONP1chr19

5705783

-DUS3Lchr19

5790346

-
In-frameENST00000360614ENST00000309061LONP1chr19

5705782

-DUS3Lchr19

5790346

-
In-frameENST00000360614ENST00000320699LONP1chr19

5705783

-DUS3Lchr19

5790346

-
In-frameENST00000360614ENST00000320699LONP1chr19

5705782

-DUS3Lchr19

5790346

-
In-frameENST00000540670ENST00000309061LONP1chr19

5705783

-DUS3Lchr19

5790346

-
In-frameENST00000540670ENST00000309061LONP1chr19

5705782

-DUS3Lchr19

5790346

-
In-frameENST00000540670ENST00000320699LONP1chr19

5705783

-DUS3Lchr19

5790346

-
In-frameENST00000540670ENST00000320699LONP1chr19

5705782

-DUS3Lchr19

5790346

-
In-frameENST00000590729ENST00000309061LONP1chr19

5705783

-DUS3Lchr19

5790346

-
In-frameENST00000590729ENST00000309061LONP1chr19

5705782

-DUS3Lchr19

5790346

-
In-frameENST00000590729ENST00000320699LONP1chr19

5705783

-DUS3Lchr19

5790346

-
In-frameENST00000590729ENST00000320699LONP1chr19

5705782

-DUS3Lchr19

5790346

-
In-frameENST00000593119ENST00000309061LONP1chr19

5705783

-DUS3Lchr19

5790346

-
In-frameENST00000593119ENST00000309061LONP1chr19

5705782

-DUS3Lchr19

5790346

-
In-frameENST00000593119ENST00000320699LONP1chr19

5705783

-DUS3Lchr19

5790346

-
In-frameENST00000593119ENST00000320699LONP1chr19

5705782

-DUS3Lchr19

5790346

-
intron-3CDSENST00000590511ENST00000309061LONP1chr19

5705783

-DUS3Lchr19

5790346

-
intron-3CDSENST00000590511ENST00000309061LONP1chr19

5705782

-DUS3Lchr19

5790346

-
intron-3CDSENST00000590511ENST00000320699LONP1chr19

5705783

-DUS3Lchr19

5790346

-
intron-3CDSENST00000590511ENST00000320699LONP1chr19

5705782

-DUS3Lchr19

5790346

-
intron-5UTRENST00000590511ENST00000590681LONP1chr19

5705783

-DUS3Lchr19

5790346

-
intron-5UTRENST00000590511ENST00000590681LONP1chr19

5705782

-DUS3Lchr19

5790346

-

check buttonORFfinder result based on the fusion transcript sequence of in-frame fusion genes.
HenstTenstHgeneHchrHbpHstrandTgeneTchrTbpTstrandSeq length
(transcript)		BP loci
(transcript)		Predicted start
(transcript)		Predicted stop
(transcript)		Seq length
(amino acids)

check buttonDeepORF prediction of the coding potential based on the fusion transcript sequence of in-frame fusion genes. DeepORF is a coding potential classifier based on convolutional neural network by comparing the real Ribo-seq data. If the no-coding score < 0.5 and coding score > 0.5, then the in-frame fusion transcript is predicted as being likely translated.
HenstTenstHgeneHchrHbpHstrandTgeneTchrTbpTstrandNo-coding scoreCoding score

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Fusion Genomic Features for LONP1-DUS3L


check buttonFusionAI prediction of the potential fusion gene breakpoint based on the pre-mature RNA sequence context (+/- 5kb of individual partner genes, total 20kb length sequence). FusionAI is a fusion gene breakpoint classifier based on convolutional neural network by comparing the fusion positive and negative sequence context of ~ 20K fusion gene data. From here, we can have the relative potentency of the 20K genomic sequence how individual sequnce will be likely used as the gene fusion breakpoints.
HgeneHchrHbpHstrandTgeneTchrTbpTstrand1-pp (fusion gene breakpoint)

check buttonDistribution of 44 human genomic features loci across 20kb length fusion breakpoint regions. We integrated a total of 44 different types of human genomic feature loci information across five big categories including virus integration sites, repeats, structural variants, chromatin states, and gene expression regulation. More details are in help page.
genomic feature

check buttonDistribution of 44 human genomic features loci across 20kb length fusion breakpoint regions that are ovelapped with the top 1% feature importance score regions. More details are in help page.

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Fusion Protein Features for LONP1-DUS3L


check button Four levels of functional features of fusion genes
Go to FGviewer search page for the most frequent breakpoint (https://ccsmweb.uth.edu/FGviewer/chr19:5705782/chr19:5790346)
- FGviewer provides the online visualization of the retention search of the protein functional features across DNA, RNA, protein, and pathological levels.
- How to search
1. Put your fusion gene symbol.
2. Press the tab key until there will be shown the breakpoint information filled.
4. Go down and press 'Search' tab twice.
4. Go down to have the hyperlink of the search result.
5. Click the hyperlink.
6. See the FGviewer result for your fusion gene.
FGviewer

check buttonMain function of each fusion partner protein. (from UniProt)
HgeneTgene
LONP1

P36776

DUS3L

Q96G46

FUNCTION: ATP-dependent serine protease that mediates the selective degradation of misfolded, unassembled or oxidatively damaged polypeptides as well as certain short-lived regulatory proteins in the mitochondrial matrix. May also have a chaperone function in the assembly of inner membrane protein complexes. Participates in the regulation of mitochondrial gene expression and in the maintenance of the integrity of the mitochondrial genome. Binds to mitochondrial promoters and RNA in a single-stranded, site-specific, and strand-specific manner. May regulate mitochondrial DNA replication and/or gene expression using site-specific, single-stranded DNA binding to target the degradation of regulatory proteins binding to adjacent sites in mitochondrial promoters (PubMed:12198491, PubMed:15870080, PubMed:17420247, PubMed:8248235). Endogenous substrates include mitochondrial steroidogenic acute regulatory (StAR) protein, helicase Twinkle (TWNK) and the large ribosomal subunit protein bL32m. bL32m is protected from degradation by LONP1 when it is bound to a nucleic acid (RNA), but TWNK is not (PubMed:17579211, PubMed:28377575). {ECO:0000255|HAMAP-Rule:MF_03120, ECO:0000269|PubMed:12198491, ECO:0000269|PubMed:15870080, ECO:0000269|PubMed:17420247, ECO:0000269|PubMed:17579211, ECO:0000269|PubMed:28377575, ECO:0000269|PubMed:8248235}.FUNCTION: Catalyzes the synthesis of dihydrouridine, a modified base found in the D-loop of most tRNAs. {ECO:0000250}.

check buttonRetention analysis result of each fusion partner protein across 39 protein features of UniProt such as six molecule processing features, 13 region features, four site features, six amino acid modification features, two natural variation features, five experimental info features, and 3 secondary structure features. Here, because of limited space for viewing, we only show the protein feature retention information belong to the 13 regional features. All retention annotation result can be downloaded at

download page


* Minus value of BPloci means that the break pointn is located before the CDS.
- In-frame and retained protein feature among the 13 regional features.
PartnerGeneHbpTbpENSTStrandBPexonTotalExonProtein feature loci*BPlociTotalLenProtein featureProtein feature note
HgeneLONP1chr19:5705782chr19:5790346ENST00000360614-818124_368455960.0DomainLon N-terminal
HgeneLONP1chr19:5705783chr19:5790346ENST00000360614-818124_368455960.0DomainLon N-terminal
TgeneDUS3Lchr19:5705782chr19:5790346ENST00000309061013311_31332651.0Nucleotide bindingFMN
TgeneDUS3Lchr19:5705782chr19:5790346ENST00000309061013497_49932651.0Nucleotide bindingFMN
TgeneDUS3Lchr19:5705782chr19:5790346ENST00000309061013520_52132651.0Nucleotide bindingFMN
TgeneDUS3Lchr19:5705782chr19:5790346ENST00000320699012311_31332409.0Nucleotide bindingFMN
TgeneDUS3Lchr19:5705782chr19:5790346ENST00000320699012497_49932409.0Nucleotide bindingFMN
TgeneDUS3Lchr19:5705782chr19:5790346ENST00000320699012520_52132409.0Nucleotide bindingFMN
TgeneDUS3Lchr19:5705783chr19:5790346ENST00000309061013311_31332651.0Nucleotide bindingFMN
TgeneDUS3Lchr19:5705783chr19:5790346ENST00000309061013497_49932651.0Nucleotide bindingFMN
TgeneDUS3Lchr19:5705783chr19:5790346ENST00000309061013520_52132651.0Nucleotide bindingFMN
TgeneDUS3Lchr19:5705783chr19:5790346ENST00000320699012311_31332409.0Nucleotide bindingFMN
TgeneDUS3Lchr19:5705783chr19:5790346ENST00000320699012497_49932409.0Nucleotide bindingFMN
TgeneDUS3Lchr19:5705783chr19:5790346ENST00000320699012520_52132409.0Nucleotide bindingFMN
TgeneDUS3Lchr19:5705782chr19:5790346ENST00000309061013118_14832651.0Zinc fingerC3H1-type 1
TgeneDUS3Lchr19:5705782chr19:5790346ENST00000309061013156_18632651.0Zinc fingerC3H1-type 2
TgeneDUS3Lchr19:5705782chr19:5790346ENST00000320699012118_14832409.0Zinc fingerC3H1-type 1
TgeneDUS3Lchr19:5705782chr19:5790346ENST00000320699012156_18632409.0Zinc fingerC3H1-type 2
TgeneDUS3Lchr19:5705783chr19:5790346ENST00000309061013118_14832651.0Zinc fingerC3H1-type 1
TgeneDUS3Lchr19:5705783chr19:5790346ENST00000309061013156_18632651.0Zinc fingerC3H1-type 2
TgeneDUS3Lchr19:5705783chr19:5790346ENST00000320699012118_14832409.0Zinc fingerC3H1-type 1
TgeneDUS3Lchr19:5705783chr19:5790346ENST00000320699012156_18632409.0Zinc fingerC3H1-type 2

- In-frame and not-retained protein feature among the 13 regional features.
PartnerGeneHbpTbpENSTStrandBPexonTotalExonProtein feature loci*BPlociTotalLenProtein featureProtein feature note
HgeneLONP1chr19:5705782chr19:5790346ENST00000360614-818759_949455960.0DomainLon proteolytic
HgeneLONP1chr19:5705783chr19:5790346ENST00000360614-818759_949455960.0DomainLon proteolytic
HgeneLONP1chr19:5705782chr19:5790346ENST00000360614-818523_530455960.0Nucleotide bindingATP
HgeneLONP1chr19:5705783chr19:5790346ENST00000360614-818523_530455960.0Nucleotide bindingATP


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Fusion Gene Sequence for LONP1-DUS3L


check button For in-frame fusion transcripts, we provide the fusion transcript sequences and fusion amino acid sequences. To have fusion amino acid sequence, we ran ORFfinder and chose the longest ORF among the all predicted ones.

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Fusion Gene PPI Analysis for LONP1-DUS3L


check button Go to ChiPPI (Chimeric Protein-Protein interactions) to see the chimeric PPI interaction in

ChiPPI page.


check button Protein-protein interactors with each fusion partner protein in wild-type (BIOGRID-3.4.160)
HgeneHgene's interactorsTgeneTgene's interactors


check button - Retained PPIs in in-frame fusion.
PartnerGeneHbpTbpENSTStrandBPexonTotalExonProtein feature loci*BPlociTotalLenStill interaction with


check button - Lost PPIs in in-frame fusion.
PartnerGeneHbpTbpENSTStrandBPexonTotalExonProtein feature loci*BPlociTotalLenInteraction lost with


check button - Retained PPIs, but lost function due to frame-shift fusion.
PartnerGeneHbpTbpENSTStrandBPexonTotalExonProtein feature loci*BPlociTotalLenInteraction lost with


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Related Drugs for LONP1-DUS3L


check button Drugs targeting genes involved in this fusion gene.
(DrugBank Version 5.1.8 2021-05-08)
PartnerGeneUniProtAccDrugBank IDDrug nameDrug activityDrug typeDrug status

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Related Diseases for LONP1-DUS3L


check button Diseases associated with fusion partners.
(DisGeNet 4.0)
PartnerGeneDisease IDDisease name# pubmedsSource