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	Fusion Gene Summary
	Fusion Gene Sample Information
	Fusion ORF Analysis
	Fusion Amino Acid Sequences
	Fusion Protein Functional Features
	Fusion Protein Structure
	pLDDT scores
	Ramachandran Plot of Fusion Protein Structure
	Fusion Protein-Protein Interaction
	Related drugs with this fusion protein
	Related disease with this fusion protein

Fusion Protein:OPA1-PPP2R3A

Fusion Protein Summary

Fusion gene summary

Fusion partner gene information	Fusion gene name: OPA1-PPP2R3A
	FusionPDB ID: 61527
	FusionGDB2.0 ID: 61527
		Hgene	Tgene
	Gene symbol	OPA1	PPP2R3A
	Gene ID	9968	5523
	Gene name	mediator complex subunit 12	protein phosphatase 2 regulatory subunit B''alpha
	Synonyms	ARC240\|CAGH45\|FGS1\|HOPA\|Kto\|MED12S\|OHDOX\|OKS\|OPA1\|TNRC11\|TRAP230	PPP2R3\|PR130\|PR72
	Cytomap	Xq13.1	3q22.2-q22.3
	Type of gene	protein-coding	protein-coding
	Description	mediator of RNA polymerase II transcription subunit 12CAG repeat protein 45Kohtalo homologOPA-containing proteinactivator-recruited cofactor 240 kDa componenthuman opposite pairedmediator of RNA polymerase II transcription, subunit 12 homologputati	serine/threonine-protein phosphatase 2A regulatory subunit B'' subunit alphaPP2A subunit B isoforms B72/B130PP2A, subunit B, R3 isoformPR130/B''alpha1 subunitPR72/B''alpha2 subuitprotein phosphatase 2 (formerly 2A), regulatory subunit B'' (PR 72), al
	Modification date	20200321	20200313
	UniProtAcc	.	.
Ensembl transtripts involved in fusion gene	ENST ids	ENST00000361150, ENST00000361510, ENST00000361715, ENST00000361828, ENST00000361908, ENST00000392438, ENST00000487986,	ENST00000469270, ENST00000492624, ENST00000264977, ENST00000334546, ENST00000490467,
Fusion gene scores for assessment (based on all fusion genes of FusionGDB 2.0)	* DoF score	5 X 3 X 4=60	8 X 6 X 5=240
	# samples	5	9
	** MAII score	log2(5/60*10)=-0.263034405833794 possibly effective Gene in Pan-Cancer Fusion Genes (peGinPCFGs). DoF>8 and MAII<0	log2(9/240*10)=-1.41503749927884 possibly effective Gene in Pan-Cancer Fusion Genes (peGinPCFGs). DoF>8 and MAII<0
Context (manual curation of fusion genes in FusionPDB)	PubMed: OPA1 [Title/Abstract] AND PPP2R3A [Title/Abstract] AND fusion [Title/Abstract]
Most frequent breakpoint (based on all fusion genes of FusionGDB 2.0)	OPA1(193385069)-PPP2R3A(135863885), # samples:3
Anticipated loss of major functional domain due to fusion event.	OPA1-PPP2R3A seems lost the major protein functional domain in Hgene partner, which is a CGC by not retaining the major functional domain in the partially deleted in-frame ORF. OPA1-PPP2R3A seems lost the major protein functional domain in Hgene partner, which is a CGC by not retaining the major functional domain in the partially deleted in-frame ORF. OPA1-PPP2R3A seems lost the major protein functional domain in Hgene partner, which is a essential gene by not retaining the major functional domain in the partially deleted in-frame ORF. OPA1-PPP2R3A seems lost the major protein functional domain in Hgene partner, which is a essential gene by not retaining the major functional domain in the partially deleted in-frame ORF. OPA1-PPP2R3A seems lost the major protein functional domain in Hgene partner, which is a essential gene due to the frame-shifted ORF. OPA1-PPP2R3A seems lost the major protein functional domain in Tgene 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)

Gene ontology of each fusion partner gene with evidence of Inferred from Direct Assay (IDA) from Entrez

Partner	Gene	GO ID	GO term	PubMed ID
Hgene	OPA1	GO:0006367	transcription initiation from RNA polymerase II promoter	12218053
Hgene	OPA1	GO:0045893	positive regulation of transcription, DNA-templated	10198638
Hgene	OPA1	GO:0045944	positive regulation of transcription by RNA polymerase II	12037571

Fusion gene breakpoints across OPA1 (5'-gene)
* Click on the image to open the UCSC genome browser with custom track showing this image in a new window.

Fusion gene breakpoints across PPP2R3A (3'-gene)
* Click on the image to open the UCSC genome browser with custom track showing this image in a new window.

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Fusion Gene Sample Information

Fusion gene information from FusionGDB2.0.

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.

Source	Disease	Sample	Hgene	Hchr	Hbp	Hstrand	Tgene	Tchr	Tbp	Tstrand
ChimerDB4	LUSC	TCGA-94-7943-01A	OPA1	chr3	193385069	-	PPP2R3A	chr3	135863885	+
ChimerDB4	LUSC	TCGA-94-7943-01A	OPA1	chr3	193385069	+	PPP2R3A	chr3	135863885	+
ChimerDB4	LUSC	TCGA-94-7943	OPA1	chr3	193385069	+	PPP2R3A	chr3	135863885	+

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Fusion ORF Analysis

Fusion information from ORFfinder translation from full-length transcript sequence from FusionPDB.

Henst	Tenst	Hgene	Hchr	Hbp	Hstrand	Tgene	Tchr	Tbp	Tstrand	Seq length (transcript)	BP loci (transcript)	Predicted start (transcript)	Predicted stop (transcript)	Seq length (amino acids)
ENST00000361828	OPA1	chr3	193385069	+	ENST00000490467	PPP2R3A	chr3	135863885	+	3448	3106	45	3176	1043
ENST00000361510	OPA1	chr3	193385069	+	ENST00000490467	PPP2R3A	chr3	135863885	+	3559	3217	45	3287	1080
ENST00000361150	OPA1	chr3	193385069	+	ENST00000490467	PPP2R3A	chr3	135863885	+	3397	3055	45	3125	1026
ENST00000361715	OPA1	chr3	193385069	+	ENST00000490467	PPP2R3A	chr3	135863885	+	3451	3109	45	3179	1044
ENST00000361908	OPA1	chr3	193385069	+	ENST00000490467	PPP2R3A	chr3	135863885	+	3505	3163	45	3233	1062
ENST00000392438	OPA1	chr3	193385069	+	ENST00000490467	PPP2R3A	chr3	135863885	+	3394	3052	45	3122	1025

DeepORF 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.

Henst	Tenst	Hgene	Hchr	Hbp	Hstrand	Tgene	Tchr	Tbp	Tstrand	No-coding score	Coding score
ENST00000361828	ENST00000490467	OPA1	chr3	193385069	+	PPP2R3A	chr3	135863885	+	0.000357033	0.99964297
ENST00000361510	ENST00000490467	OPA1	chr3	193385069	+	PPP2R3A	chr3	135863885	+	0.000314627	0.9996854
ENST00000361150	ENST00000490467	OPA1	chr3	193385069	+	PPP2R3A	chr3	135863885	+	0.000372217	0.9996277
ENST00000361715	ENST00000490467	OPA1	chr3	193385069	+	PPP2R3A	chr3	135863885	+	0.000447286	0.99955267
ENST00000361908	ENST00000490467	OPA1	chr3	193385069	+	PPP2R3A	chr3	135863885	+	0.000522512	0.99947757
ENST00000392438	ENST00000490467	OPA1	chr3	193385069	+	PPP2R3A	chr3	135863885	+	0.000330783	0.9996692

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Fusion Amino Acid Sequences

For individual full-length fusion transcript sequence from FusionPDB, we ran ORFfinder and chose the longest ORF among the all predicted ones.

>FusionGDB ID_FusionGDB isoform ID_FGname_Hgene_Hchr_Hbp_Henst_Tgene_Tchr_Tbp_Tenst_length(fusion AA) seq_BP

>61527_61527_1_OPA1-PPP2R3A_OPA1_chr3_193385069_ENST00000361150_PPP2R3A_chr3_135863885_ENST00000490467_length(amino acids)=1026AA_BP=1004
MSTGACQALAEVHAPLGGPRPRLCALAAEGHFLGHSWTGSRAGAHTGAPAWPSRRLRDLPAGGMWRLRRAAVACEVCQSLVKHSSGIKGS
LPLQKLHLVSRSIYHSHHPTLKLQRPQLRTSFQQFSSLTNLPLRKLKFSPIKYGYQPRRNFWPARLATRLLKLRYLILGSAVGGGYTAKK
TFDQWKDMIPDLSEYKWIVPDIVWEIDEYIDFGSPEETAFRATDRGSESDKHFRKGLLGELILLQQQIQEHEEEARRAAGQYSTSYAQQK
RKVSDKEKIDQLQEELLHTQLKYQRILERLEKENKELRKLVLQKDDKGIHHRKLKKSLIDMYSEVLDVLSDYDASYNTQDHLPRVVVVGD
QSAGKTSVLEMIAQARIFPRGSGEMMTRSPVKVTLSEGPHHVALFKDSSREFDLTKEEDLAALRHEIELRMRKNVKEGCTVSPETISLNV
KGPGLQRMVLVDLPGVINTVTSGMAPDTKETIFSISKAYMQNPNAIILCIQDGSVDAERSIVTDLVSQMDPHGRRTIFVLTKVDLAEKNV
ASPSRIQQIIEGKLFPMKALGYFAVVTGKGNSSESIEAIREYEEEFFQNSKLLKTSMLKAHQVTTRNLSLAVSDCFWKMVRESVEQQADS
FKATRFNLETEWKNNYPRLRELDRNELFEKAKNEILDEVISLSQVTPKHWEEILQQSLWERVSTHVIENIYLPAAQTMNSGTFNTTVDIK
LKQWTDKQLPNKAVEVAWETLQEEFSRFMTEPKGKEHDDIFDKLKEAVKEESIKRHKWNDFAEDSLRVIQHNALEDRSISDKQQWDAAIY
FMEEALQARLKDTENAIENMVGPDWKKRWLYWKNRTQEQCVHNETKNELEKMLKCNEEHPAYLASDEITTVRKNLESRGVEVDPSLIKDT
WHQVYRRHFLKTALNHCNLCRRGFYYYQRHFVDSELECNDVVLFWRIQRMLAITANTLRQQLTNTEVRRLEKNVKEVLEDFAEDGEKKIK

--------------------------------------------------------------

>61527_61527_2_OPA1-PPP2R3A_OPA1_chr3_193385069_ENST00000361510_PPP2R3A_chr3_135863885_ENST00000490467_length(amino acids)=1080AA_BP=1058
MSTGACQALAEVHAPLGGPRPRLCALAAEGHFLGHSWTGSRAGAHTGAPAWPSRRLRDLPAGGMWRLRRAAVACEVCQSLVKHSSGIKGS
LPLQKLHLVSRSIYHSHHPTLKLQRPQLRTSFQQFSSLTNLPLRKLKFSPIKYGYQPRRNFWPARLATRLLKLRYLILGSAVGGGYTAKK
TFDQWKDMIPDLSEYKWIVPDIVWEIDEYIDFEKIRKALPSSEDLVKLAPDFDKIVESLSLLKDFFTSGHKLVSEVIGASDLLLLLGSPE
ETAFRATDRGSESDKHFRKGLLGELILLQQQIQEHEEEARRAAGQYSTSYAQQKRKVSDKEKIDQLQEELLHTQLKYQRILERLEKENKE
LRKLVLQKDDKGIHHRKLKKSLIDMYSEVLDVLSDYDASYNTQDHLPRVVVVGDQSAGKTSVLEMIAQARIFPRGSGEMMTRSPVKVTLS
EGPHHVALFKDSSREFDLTKEEDLAALRHEIELRMRKNVKEGCTVSPETISLNVKGPGLQRMVLVDLPGVINTVTSGMAPDTKETIFSIS
KAYMQNPNAIILCIQDGSVDAERSIVTDLVSQMDPHGRRTIFVLTKVDLAEKNVASPSRIQQIIEGKLFPMKALGYFAVVTGKGNSSESI
EAIREYEEEFFQNSKLLKTSMLKAHQVTTRNLSLAVSDCFWKMVRESVEQQADSFKATRFNLETEWKNNYPRLRELDRNELFEKAKNEIL
DEVISLSQVTPKHWEEILQQSLWERVSTHVIENIYLPAAQTMNSGTFNTTVDIKLKQWTDKQLPNKAVEVAWETLQEEFSRFMTEPKGKE
HDDIFDKLKEAVKEESIKRHKWNDFAEDSLRVIQHNALEDRSISDKQQWDAAIYFMEEALQARLKDTENAIENMVGPDWKKRWLYWKNRT
QEQCVHNETKNELEKMLKCNEEHPAYLASDEITTVRKNLESRGVEVDPSLIKDTWHQVYRRHFLKTALNHCNLCRRGFYYYQRHFVDSEL
ECNDVVLFWRIQRMLAITANTLRQQLTNTEVRRLEKNVKEVLEDFAEDGEKKIKLLTGKRVQLAEDLTLKIMKQMNLPLPLNLETKAIKY

--------------------------------------------------------------

>61527_61527_3_OPA1-PPP2R3A_OPA1_chr3_193385069_ENST00000361715_PPP2R3A_chr3_135863885_ENST00000490467_length(amino acids)=1044AA_BP=1022
MSTGACQALAEVHAPLGGPRPRLCALAAEGHFLGHSWTGSRAGAHTGAPAWPSRRLRDLPAGGMWRLRRAAVACEVCQSLVKHSSGIKGS
LPLQKLHLVSRSIYHSHHPTLKLQRPQLRTSFQQFSSLTNLPLRKLKFSPIKYGYQPRRNFWPARLATRLLKLRYLILGSAVGGGYTAKK
TFDQWKDMIPDLSEYKWIVPDIVWEIDEYIDFGHKLVSEVIGASDLLLLLGSPEETAFRATDRGSESDKHFRKGLLGELILLQQQIQEHE
EEARRAAGQYSTSYAQQKRKVSDKEKIDQLQEELLHTQLKYQRILERLEKENKELRKLVLQKDDKGIHHRKLKKSLIDMYSEVLDVLSDY
DASYNTQDHLPRVVVVGDQSAGKTSVLEMIAQARIFPRGSGEMMTRSPVKVTLSEGPHHVALFKDSSREFDLTKEEDLAALRHEIELRMR
KNVKEGCTVSPETISLNVKGPGLQRMVLVDLPGVINTVTSGMAPDTKETIFSISKAYMQNPNAIILCIQDGSVDAERSIVTDLVSQMDPH
GRRTIFVLTKVDLAEKNVASPSRIQQIIEGKLFPMKALGYFAVVTGKGNSSESIEAIREYEEEFFQNSKLLKTSMLKAHQVTTRNLSLAV
SDCFWKMVRESVEQQADSFKATRFNLETEWKNNYPRLRELDRNELFEKAKNEILDEVISLSQVTPKHWEEILQQSLWERVSTHVIENIYL
PAAQTMNSGTFNTTVDIKLKQWTDKQLPNKAVEVAWETLQEEFSRFMTEPKGKEHDDIFDKLKEAVKEESIKRHKWNDFAEDSLRVIQHN
ALEDRSISDKQQWDAAIYFMEEALQARLKDTENAIENMVGPDWKKRWLYWKNRTQEQCVHNETKNELEKMLKCNEEHPAYLASDEITTVR
KNLESRGVEVDPSLIKDTWHQVYRRHFLKTALNHCNLCRRGFYYYQRHFVDSELECNDVVLFWRIQRMLAITANTLRQQLTNTEVRRLEK

--------------------------------------------------------------

>61527_61527_4_OPA1-PPP2R3A_OPA1_chr3_193385069_ENST00000361828_PPP2R3A_chr3_135863885_ENST00000490467_length(amino acids)=1043AA_BP=1021
MSTGACQALAEVHAPLGGPRPRLCALAAEGHFLGHSWTGSRAGAHTGAPAWPSRRLRDLPAGGMWRLRRAAVACEVCQSLVKHSSGIKGS
LPLQKLHLVSRSIYHSHHPTLKLQRPQLRTSFQQFSSLTNLPLRKLKFSPIKYGYQPRRNFWPARLATRLLKLRYLILGSAVGGGYTAKK
TFDQWKDMIPDLSEYKWIVPDIVWEIDEYIDFEKIRKALPSSEDLVKLAPDFDKIVESLSLLKDFFTSGHKLVSEVIGASDLLLLLGSPE
ETAFRATDRGSESDKHFRKVSDKEKIDQLQEELLHTQLKYQRILERLEKENKELRKLVLQKDDKGIHHRKLKKSLIDMYSEVLDVLSDYD
ASYNTQDHLPRVVVVGDQSAGKTSVLEMIAQARIFPRGSGEMMTRSPVKVTLSEGPHHVALFKDSSREFDLTKEEDLAALRHEIELRMRK
NVKEGCTVSPETISLNVKGPGLQRMVLVDLPGVINTVTSGMAPDTKETIFSISKAYMQNPNAIILCIQDGSVDAERSIVTDLVSQMDPHG
RRTIFVLTKVDLAEKNVASPSRIQQIIEGKLFPMKALGYFAVVTGKGNSSESIEAIREYEEEFFQNSKLLKTSMLKAHQVTTRNLSLAVS
DCFWKMVRESVEQQADSFKATRFNLETEWKNNYPRLRELDRNELFEKAKNEILDEVISLSQVTPKHWEEILQQSLWERVSTHVIENIYLP
AAQTMNSGTFNTTVDIKLKQWTDKQLPNKAVEVAWETLQEEFSRFMTEPKGKEHDDIFDKLKEAVKEESIKRHKWNDFAEDSLRVIQHNA
LEDRSISDKQQWDAAIYFMEEALQARLKDTENAIENMVGPDWKKRWLYWKNRTQEQCVHNETKNELEKMLKCNEEHPAYLASDEITTVRK
NLESRGVEVDPSLIKDTWHQVYRRHFLKTALNHCNLCRRGFYYYQRHFVDSELECNDVVLFWRIQRMLAITANTLRQQLTNTEVRRLEKN

--------------------------------------------------------------

>61527_61527_5_OPA1-PPP2R3A_OPA1_chr3_193385069_ENST00000361908_PPP2R3A_chr3_135863885_ENST00000490467_length(amino acids)=1062AA_BP=1040
MSTGACQALAEVHAPLGGPRPRLCALAAEGHFLGHSWTGSRAGAHTGAPAWPSRRLRDLPAGGMWRLRRAAVACEVCQSLVKHSSGIKGS
LPLQKLHLVSRSIYHSHHPTLKLQRPQLRTSFQQFSSLTNLPLRKLKFSPIKYGYQPRRNFWPARLATRLLKLRYLILGSAVGGGYTAKK
TFDQWKDMIPDLSEYKWIVPDIVWEIDEYIDFEKIRKALPSSEDLVKLAPDFDKIVESLSLLKDFFTSGSPEETAFRATDRGSESDKHFR
KGLLGELILLQQQIQEHEEEARRAAGQYSTSYAQQKRKVSDKEKIDQLQEELLHTQLKYQRILERLEKENKELRKLVLQKDDKGIHHRKL
KKSLIDMYSEVLDVLSDYDASYNTQDHLPRVVVVGDQSAGKTSVLEMIAQARIFPRGSGEMMTRSPVKVTLSEGPHHVALFKDSSREFDL
TKEEDLAALRHEIELRMRKNVKEGCTVSPETISLNVKGPGLQRMVLVDLPGVINTVTSGMAPDTKETIFSISKAYMQNPNAIILCIQDGS
VDAERSIVTDLVSQMDPHGRRTIFVLTKVDLAEKNVASPSRIQQIIEGKLFPMKALGYFAVVTGKGNSSESIEAIREYEEEFFQNSKLLK
TSMLKAHQVTTRNLSLAVSDCFWKMVRESVEQQADSFKATRFNLETEWKNNYPRLRELDRNELFEKAKNEILDEVISLSQVTPKHWEEIL
QQSLWERVSTHVIENIYLPAAQTMNSGTFNTTVDIKLKQWTDKQLPNKAVEVAWETLQEEFSRFMTEPKGKEHDDIFDKLKEAVKEESIK
RHKWNDFAEDSLRVIQHNALEDRSISDKQQWDAAIYFMEEALQARLKDTENAIENMVGPDWKKRWLYWKNRTQEQCVHNETKNELEKMLK
CNEEHPAYLASDEITTVRKNLESRGVEVDPSLIKDTWHQVYRRHFLKTALNHCNLCRRGFYYYQRHFVDSELECNDVVLFWRIQRMLAIT

--------------------------------------------------------------

>61527_61527_6_OPA1-PPP2R3A_OPA1_chr3_193385069_ENST00000392438_PPP2R3A_chr3_135863885_ENST00000490467_length(amino acids)=1025AA_BP=1003
MSTGACQALAEVHAPLGGPRPRLCALAAEGHFLGHSWTGSRAGAHTGAPAWPSRRLRDLPAGGMWRLRRAAVACEVCQSLVKHSSGIKGS
LPLQKLHLVSRSIYHSHHPTLKLQRPQLRTSFQQFSSLTNLPLRKLKFSPIKYGYQPRRNFWPARLATRLLKLRYLILGSAVGGGYTAKK
TFDQWKDMIPDLSEYKWIVPDIVWEIDEYIDFEKIRKALPSSEDLVKLAPDFDKIVESLSLLKDFFTSGSPEETAFRATDRGSESDKHFR
KVSDKEKIDQLQEELLHTQLKYQRILERLEKENKELRKLVLQKDDKGIHHRKLKKSLIDMYSEVLDVLSDYDASYNTQDHLPRVVVVGDQ
SAGKTSVLEMIAQARIFPRGSGEMMTRSPVKVTLSEGPHHVALFKDSSREFDLTKEEDLAALRHEIELRMRKNVKEGCTVSPETISLNVK
GPGLQRMVLVDLPGVINTVTSGMAPDTKETIFSISKAYMQNPNAIILCIQDGSVDAERSIVTDLVSQMDPHGRRTIFVLTKVDLAEKNVA
SPSRIQQIIEGKLFPMKALGYFAVVTGKGNSSESIEAIREYEEEFFQNSKLLKTSMLKAHQVTTRNLSLAVSDCFWKMVRESVEQQADSF
KATRFNLETEWKNNYPRLRELDRNELFEKAKNEILDEVISLSQVTPKHWEEILQQSLWERVSTHVIENIYLPAAQTMNSGTFNTTVDIKL
KQWTDKQLPNKAVEVAWETLQEEFSRFMTEPKGKEHDDIFDKLKEAVKEESIKRHKWNDFAEDSLRVIQHNALEDRSISDKQQWDAAIYF
MEEALQARLKDTENAIENMVGPDWKKRWLYWKNRTQEQCVHNETKNELEKMLKCNEEHPAYLASDEITTVRKNLESRGVEVDPSLIKDTW
HQVYRRHFLKTALNHCNLCRRGFYYYQRHFVDSELECNDVVLFWRIQRMLAITANTLRQQLTNTEVRRLEKNVKEVLEDFAEDGEKKIKL

--------------------------------------------------------------

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Fusion Protein Functional Features

Four levels of functional features of fusion genes
Go to FGviewer search page for the most frequent breakpoint (https://ccsmweb.uth.edu/FGviewer/chr3:193385069/chr3:135863885)
- 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.

Main function of each fusion partner protein. (from UniProt)

Hgene	Tgene
.	.
FUNCTION: Might normally function as a transcriptional repressor. EWS-fusion-proteins (EFPS) may play a role in the tumorigenic process. They may disturb gene expression by mimicking, or interfering with the normal function of CTD-POLII within the transcription initiation complex. They may also contribute to an aberrant activation of the fusion protein target genes.	FUNCTION: Might normally function as a transcriptional repressor. EWS-fusion-proteins (EFPS) may play a role in the tumorigenic process. They may disturb gene expression by mimicking, or interfering with the normal function of CTD-POLII within the transcription initiation complex. They may also contribute to an aberrant activation of the fusion protein target genes.

Retention 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.

- Retained protein feature among the 13 regional features.

Partner

Gene

Hbp

Tbp

ENST

Strand

BPexon

TotalExon

Protein feature loci

*BPloci

TotalLen

Protein feature

Protein feature note

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000361908

210_254

976.3333333333334

1978.0

Coiled coil

Ontology_term=ECO:0000255

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000361908

895_960

976.3333333333334

1978.0

Coiled coil

Ontology_term=ECO:0000255

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000392438

210_254

939.3333333333334

1945.0

Coiled coil

Ontology_term=ECO:0000255

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000361908

285_561

976.3333333333334

1978.0

Domain

Dynamin-type G

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000392438

285_561

939.3333333333334

1945.0

Domain

Dynamin-type G

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000361908

398_402

976.3333333333334

1978.0

Nucleotide binding

GTP

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000392438

398_402

939.3333333333334

1945.0

Nucleotide binding

GTP

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000361908

295_302

976.3333333333334

1978.0

Region

G1 motif

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000361908

321_324

976.3333333333334

1978.0

Region

G2 motif

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000361908

398_401

976.3333333333334

1978.0

Region

G3 motif

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000361908

467_470

976.3333333333334

1978.0

Region

G4 motif

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000361908

501_504

976.3333333333334

1978.0

Region

G5 motif

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000392438

295_302

939.3333333333334

1945.0

Region

G1 motif

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000392438

321_324

939.3333333333334

1945.0

Region

G2 motif

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000392438

398_401

939.3333333333334

1945.0

Region

G3 motif

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000392438

467_470

939.3333333333334

1945.0

Region

G4 motif

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000392438

501_504

939.3333333333334

1945.0

Region

G5 motif

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000361908

114_960

976.3333333333334

1978.0

Topological domain

Mitochondrial intermembrane

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000361908

88_96

976.3333333333334

1978.0

Topological domain

Mitochondrial matrix

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000392438

88_96

939.3333333333334

1945.0

Topological domain

Mitochondrial matrix

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000361908

97_113

976.3333333333334

1978.0

Transmembrane

Helical

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000392438

97_113

939.3333333333334

1945.0

Transmembrane

Helical

Tgene

PPP2R3A

chr3:193385069

chr3:135863885

ENST00000334546

985_996

488.6666666666667

530.0

Calcium binding

Ontology_term=ECO:0000255

Tgene

PPP2R3A

chr3:193385069

chr3:135863885

ENST00000490467

985_996

373.6666666666667

415.0

Calcium binding

Ontology_term=ECO:0000255

Tgene

PPP2R3A

chr3:193385069

chr3:135863885

ENST00000334546

670_695

488.6666666666667

530.0

Compositional bias

Note=Pro-rich

Tgene

PPP2R3A

chr3:193385069

chr3:135863885

ENST00000490467

670_695

373.6666666666667

415.0

Compositional bias

Note=Pro-rich

Tgene

PPP2R3A

chr3:193385069

chr3:135863885

ENST00000334546

758_793

488.6666666666667

530.0

Domain

EF-hand 1

Tgene

PPP2R3A

chr3:193385069

chr3:135863885

ENST00000334546

972_1007

488.6666666666667

530.0

Domain

EF-hand 2

Tgene

PPP2R3A

chr3:193385069

chr3:135863885

ENST00000490467

758_793

373.6666666666667

415.0

Domain

EF-hand 1

Tgene

PPP2R3A

chr3:193385069

chr3:135863885

ENST00000490467

972_1007

373.6666666666667

415.0

Domain

EF-hand 2

- Not-retained protein feature among the 13 regional features.

Partner

Gene

Hbp

Tbp

ENST

Strand

BPexon

TotalExon

Protein feature loci

*BPloci

TotalLen

Protein feature

Protein feature note

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000392438

895_960

939.3333333333334

1945.0

Coiled coil

Ontology_term=ECO:0000255

Hgene

OPA1

chr3:193385069

chr3:135863885

ENST00000392438

114_960

939.3333333333334

1945.0

Topological domain

Mitochondrial intermembrane

Tgene

PPP2R3A

chr3:193385069

chr3:135863885

ENST00000264977

985_996

1109.6666666666667

1151.0

Calcium binding

Ontology_term=ECO:0000255

Tgene

PPP2R3A

chr3:193385069

chr3:135863885

ENST00000264977

670_695

1109.6666666666667

1151.0

Compositional bias

Note=Pro-rich

Tgene

PPP2R3A

chr3:193385069

chr3:135863885

ENST00000264977

758_793

1109.6666666666667

1151.0

Domain

EF-hand 1

Tgene

PPP2R3A

chr3:193385069

chr3:135863885

ENST00000264977

972_1007

1109.6666666666667

1151.0

Domain

EF-hand 2

Top

Fusion Protein Structures

PDB and CIF files of the predicted fusion proteins
* Here we show the 3D structure of the fusion proteins using Mol*. AlphaFold produces a per-residue confidence score (pLDDT) between 0 and 100. Model confidence is shown from the pLDDT values per residue. pLDDT corresponds to the model’s prediction of its score on the local Distance Difference Test. It is a measure of local accuracy (from AlphfaFold website). To color code individual residues, we transformed individual PDB files into CIF format.

Fusion protein PDB link (fusion AA seq ID in FusionPDB)	Hgene	Hchr	Hbp	Hstrand	Tgene	Tchr	Tbp	Tstrand	AA seq	Len(AA seq)
PDB file >>>1849_OPA1_193385069_PPP2R3A_135863885_ranked_0.pdb	OPA1	193385069	193385069	ENST00000490467	PPP2R3A	chr3	135863885	+	MSTGACQALAEVHAPLGGPRPRLCALAAEGHFLGHSWTGSRAGAHTGAPAWPSRRLRDLPAGGMWRLRRAAVACEVCQSLVKHSSGIKGS LPLQKLHLVSRSIYHSHHPTLKLQRPQLRTSFQQFSSLTNLPLRKLKFSPIKYGYQPRRNFWPARLATRLLKLRYLILGSAVGGGYTAKK TFDQWKDMIPDLSEYKWIVPDIVWEIDEYIDFEKIRKALPSSEDLVKLAPDFDKIVESLSLLKDFFTSGHKLVSEVIGASDLLLLLGSPE ETAFRATDRGSESDKHFRKGLLGELILLQQQIQEHEEEARRAAGQYSTSYAQQKRKVSDKEKIDQLQEELLHTQLKYQRILERLEKENKE LRKLVLQKDDKGIHHRKLKKSLIDMYSEVLDVLSDYDASYNTQDHLPRVVVVGDQSAGKTSVLEMIAQARIFPRGSGEMMTRSPVKVTLS EGPHHVALFKDSSREFDLTKEEDLAALRHEIELRMRKNVKEGCTVSPETISLNVKGPGLQRMVLVDLPGVINTVTSGMAPDTKETIFSIS KAYMQNPNAIILCIQDGSVDAERSIVTDLVSQMDPHGRRTIFVLTKVDLAEKNVASPSRIQQIIEGKLFPMKALGYFAVVTGKGNSSESI EAIREYEEEFFQNSKLLKTSMLKAHQVTTRNLSLAVSDCFWKMVRESVEQQADSFKATRFNLETEWKNNYPRLRELDRNELFEKAKNEIL DEVISLSQVTPKHWEEILQQSLWERVSTHVIENIYLPAAQTMNSGTFNTTVDIKLKQWTDKQLPNKAVEVAWETLQEEFSRFMTEPKGKE HDDIFDKLKEAVKEESIKRHKWNDFAEDSLRVIQHNALEDRSISDKQQWDAAIYFMEEALQARLKDTENAIENMVGPDWKKRWLYWKNRT QEQCVHNETKNELEKMLKCNEEHPAYLASDEITTVRKNLESRGVEVDPSLIKDTWHQVYRRHFLKTALNHCNLCRRGFYYYQRHFVDSEL ECNDVVLFWRIQRMLAITANTLRQQLTNTEVRRLEKNVKEVLEDFAEDGEKKIKLLTGKRVQLAEDLTLKIMKQMNLPLPLNLETKAIKY	1080

Top

pLDDT score distribution

pLDDT score distribution of the predicted wild-type structures of two partner proteins from AlphaFold2
* AlphaFold produces a per-residue confidence score (pLDDT) between 0 and 100.

OPA1_pLDDT.png

PPP2R3A_pLDDT.png

pLDDT score distribution of the predicted fusion protein structures from AlphaFold2
* AlphaFold produces a per-residue confidence score (pLDDT) between 0 and 100.

Top

Ramachandran Plot of Fusion Protein Structure

Ramachandran plot of the torsional angles - phi (φ)and psi (ψ) - of the residues (amino acids) contained in this fusion protein peptide.

Fusion AA seq ID in FusionPDB and their Ramachandran plots

Top

Fusion Protein-Protein Interaction

Go to ChiPPI (Chimeric Protein-Protein interactions) to see the chimeric PPI interaction in
ChiPPI page.

Protein-protein interactors with each fusion partner protein in wild-type from validated records (BIOGRID-3.4.160)

Gene

PPI interactors

Protein-protein interactors based on sequence similarity (STRING)

Gene	STRING network
OPA1
PPP2R3A

- Retained interactions in fusion protein (protein functional feature from UniProt).

Partner

Gene

Hbp

Tbp

ENST

Strand

BPexon

TotalExon

Protein feature loci

*BPloci

TotalLen

Still interaction with

- Lost interactions due to fusion (protein functional feature from UniProt).

Partner

Gene

Hbp

Tbp

ENST

Strand

BPexon

TotalExon

Protein feature loci

*BPloci

TotalLen

Interaction lost with

Top

Related Drugs to OPA1-PPP2R3A

Drugs used for this fusion-positive patient.
(Manual curation of PubMed, 04-30-2022 + MyCancerGenome)

Hgene

Tgene

Drug

Source

PMID

Top

Related Diseases to OPA1-PPP2R3A

Diseases that have this fusion gene.
(Manual curation of PubMed, 04-30-2022 + MyCancerGenome)

Hgene

Tgene

Disease

Source

PMID

Diseases associated with fusion partners.
(DisGeNet 4.0)

Partner

Gene

Disease ID

Disease name

# pubmeds

Source