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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:ZNF276-SPG7 (FusionGDB2 ID:101068)

Fusion Gene Summary for ZNF276-SPG7

check button Fusion gene summary
Fusion gene informationFusion gene name: ZNF276-SPG7
Fusion gene ID: 101068
HgeneTgene
Gene symbol

ZNF276

SPG7

Gene ID

92822

6687

Gene namezinc finger protein 276SPG7 matrix AAA peptidase subunit, paraplegin
SynonymsCENP-Z|CENPZ|ZADT|ZFP276|ZNF477CAR|CMAR|PGN|SPG5C
Cytomap

16q24.3

16q24.3

Type of geneprotein-codingprotein-coding
Descriptionzinc finger protein 276centromere protein Zzinc finger protein 276 homologzinc finger protein 477zinc finger, AD-typeparapleginSPG7, paraplegin matrix AAA peptidase subunitcell matrix adhesion regulatorspastic paraplegia 7 (pure and complicated autosomal recessive)spastic paraplegia 7 protein
Modification date2020032020200320
UniProtAcc..
Ensembl transtripts involved in fusion geneENST00000446326, ENST00000289816, 
ENST00000568064, ENST00000443381, 
ENST00000268704, ENST00000341316, 
ENST00000565891, 
Fusion gene scores* DoF score4 X 4 X 3=4811 X 8 X 7=616
# samples 411
** MAII scorelog2(4/48*10)=-0.263034405833794
possibly effective Gene in Pan-Cancer Fusion Genes (peGinPCFGs).
DoF>8 and MAII<0
log2(11/616*10)=-2.48542682717024
possibly effective Gene in Pan-Cancer Fusion Genes (peGinPCFGs).
DoF>8 and MAII<0
Context

PubMed: ZNF276 [Title/Abstract] AND SPG7 [Title/Abstract] AND fusion [Title/Abstract]

Most frequent breakpointZNF276(89805703)-SPG7(89613066), # samples:2
Anticipated loss of major functional domain due to fusion event.
* 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

check buttonFusion gene breakpoints across ZNF276 (5'-gene)
* Click on the image to open the UCSC genome browser with custom track showing this image in a new window.

check buttonFusion gene breakpoints across SPG7 (3'-gene)
* Click on the image to open the UCSC genome browser with custom track showing this image in a new window.

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
ChimerDB4BLCATCGA-G2-A2EC-01AZNF276chr16

89805703

+SPG7chr16

89613066

+
ChimerDB4BLCATCGA-G2-A2EC-01AZNF276chr16

89805703

+SPG7chr16

89613066

+


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Fusion Gene ORF analysis for ZNF276-SPG7

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
intron-3CDSENST00000446326ENST00000268704ZNF276chr16

89805703

+SPG7chr16

89613066

+
intron-intronENST00000446326ENST00000341316ZNF276chr16

89805703

+SPG7chr16

89613066

+
intron-intronENST00000446326ENST00000565891ZNF276chr16

89805703

+SPG7chr16

89613066

+
3UTR-3CDSENST00000289816ENST00000268704ZNF276chr16

89805703

+SPG7chr16

89613066

+
3UTR-intronENST00000289816ENST00000341316ZNF276chr16

89805703

+SPG7chr16

89613066

+
3UTR-intronENST00000289816ENST00000565891ZNF276chr16

89805703

+SPG7chr16

89613066

+
intron-3CDSENST00000568064ENST00000268704ZNF276chr16

89805703

+SPG7chr16

89613066

+
intron-intronENST00000568064ENST00000341316ZNF276chr16

89805703

+SPG7chr16

89613066

+
intron-intronENST00000568064ENST00000565891ZNF276chr16

89805703

+SPG7chr16

89613066

+
intron-3CDSENST00000443381ENST00000268704ZNF276chr16

89805703

+SPG7chr16

89613066

+
intron-intronENST00000443381ENST00000341316ZNF276chr16

89805703

+SPG7chr16

89613066

+
intron-intronENST00000443381ENST00000565891ZNF276chr16

89805703

+SPG7chr16

89613066

+

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 ZNF276-SPG7


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.

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 ZNF276-SPG7


check button Go to

FGviewer for the breakpoints of :-:

.
- FGviewer provides the online visualization of the retention search of the protein functional features across DNA, RNA, protein, and pathological levels.
FGviewer

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

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

- In-frame and not-retained protein feature among the 13 regional features.
PartnerGeneHbpTbpENSTStrandBPexonTotalExonProtein feature loci*BPlociTotalLenProtein featureProtein feature note


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Fusion Gene Sequence for ZNF276-SPG7


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 ZNF276-SPG7


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 ZNF276-SPG7


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 ZNF276-SPG7


check button Diseases associated with fusion partners.
(DisGeNet 4.0)
PartnerGeneDisease IDDisease name# pubmedsSource
TgeneSPG7C1846564SPASTIC PARAPLEGIA 7, AUTOSOMAL RECESSIVE9CTD_human;GENOMICS_ENGLAND;ORPHANET;UNIPROT
TgeneSPG7C0030486Paraplegia1CTD_human
TgeneSPG7C0037772Spastic Paraplegia1CTD_human
TgeneSPG7C0154682Lateral Sclerosis1ORPHANET
TgeneSPG7C0242036Paraplegia, Ataxic1CTD_human
TgeneSPG7C0278114Paraplegia, Cerebral1CTD_human
TgeneSPG7C0278115Paraplegia, Spinal1CTD_human
TgeneSPG7C0452143Paraplegia, Flaccid1CTD_human
TgeneSPG7C1968845Primary Lateral Sclerosis, Adult, 11ORPHANET
TgeneSPG7C3711370Spastic Paraplegia Type 71ORPHANET