Mus musculus Gene: Peo1
Summary
InnateDB Gene IDBG-170422.6
Last Modified 2014-10-13 [Report errors or provide feedback]
Gene Symbol Peo1
Gene Name progressive external ophthalmoplegia 1 (human)
Synonyms
Species Mus musculus
Ensembl Gene ENSMUSG00000025209
Encoded Proteins
progressive external ophthalmoplegia 1 (human)
Protein Structure
Useful resources Stemformatics EHFPI ImmGen
Entrez Gene
Summary This gene does not have any Entrez summary - the following is the summary from its human ortholog ENSG00000107815:
This gene encodes a hexameric DNA helicase which unwinds short stretches of double-stranded DNA in the 5' to 3' direction and, along with mitochondrial single-stranded DNA binding protein and mtDNA polymerase gamma, is thought to play a key role in mtDNA replication. The protein localizes to the mitochondrial matrix and mitochondrial nucleoids. Mutations in this gene cause infantile onset spinocerebellar ataxia (IOSCA) and progressive external ophthalmoplegia (PEO) and are also associated with several mitochondrial depletion syndromes. Alternative splicing results in multiple transcript variants encoding distinct isoforms.[provided by RefSeq, Aug 2009]
Gene Information
Type Protein coding
Genomic Location Chromosome 19:45006558-45012762
Strand Forward strand
Band C3
Transcripts
ENSMUST00000026227 ENSMUSP00000026227
Interactions
Number of Interactions This gene and/or its encoded proteins are associated with 0 experimentally validated interaction(s) in this database.
They are also associated with 5 interaction(s) predicted by orthology.
Predicted by orthology
Total 5 [view]
Gene Ontology

Molecular Function
Accession GO Term
GO:0002020 protease binding
GO:0003678 DNA helicase activity
GO:0003697 single-stranded DNA binding
GO:0005524 ATP binding
GO:0043139 5'-3' DNA helicase activity
Biological Process
GO:0006260 DNA replication
GO:0006264 mitochondrial DNA replication
GO:0006268 DNA unwinding involved in DNA replication
GO:0006390 transcription from mitochondrial promoter
GO:0034214 protein hexamerization
GO:0051260 protein homooligomerization
Cellular Component
GO:0005739 mitochondrion
GO:0042645 mitochondrial nucleoid
Orthologs
Species
Homo sapiens
Bos taurus
Gene ID
Gene Order
Not yet available
Pathway Predictions based on Human Orthology Data
NETPATH
REACTOME
Organelle biogenesis and maintenance pathway
Mitochondrial biogenesis pathway
Transcriptional activation of mitochondrial biogenesis pathway
KEGG
INOH
PID NCI
Cross-References
SwissProt Q8CIW5
TrEMBL Q80WJ5
UniProt Splice Variant
Entrez Gene 226153
UniGene Mm.105585
RefSeq NM_153796 XM_006527003
OMIM
CCDS CCDS29854
HPRD
IMGT
MGI ID MGI:2137410
MGI Symbol Peo1
EMBL AF503291 AK146244 AY059385 BC034909 BC071195
GenPept AAH34909 AAH71195 AAL27647 AAP30837 BAE27008
RNA Seq Atlas 226153