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    Spg7 SPG7 matrix AAA peptidase subunit, paraplegin [ Rattus norvegicus (Norway rat) ]

    Gene ID: 353231, updated on 9-Dec-2024

    Summary

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    Official Symbol
    Spg7provided by RGD
    Official Full Name
    SPG7 matrix AAA peptidase subunit, parapleginprovided by RGD
    Primary source
    RGD:727940
    See related
    EnsemblRapid:ENSRNOG00000015150 AllianceGenome:RGD:727940
    Gene type
    protein coding
    RefSeq status
    VALIDATED
    Organism
    Rattus norvegicus
    Lineage
    Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; Murinae; Rattus
    Summary
    Predicted to enable metalloendopeptidase activity. Involved in mitochondrial outer membrane permeabilization involved in programmed cell death. Predicted to be located in mitochondrial inner membrane. Predicted to be part of m-AAA complex. Human ortholog(s) of this gene implicated in hereditary spastic paraplegia 7. Orthologous to human SPG7 (SPG7 matrix AAA peptidase subunit, paraplegin). [provided by Alliance of Genome Resources, Dec 2024]
    Expression
    Biased expression in Kidney (RPKM 134.0), Heart (RPKM 105.7) and 9 other tissues See more
    Orthologs
    human mouse all
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    Genomic context

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    See Spg7 in Genome Data Viewer
    Location:
    19q12
    Exon count:
    19
    Annotation release Status Assembly Chr Location
    RS_2024_02 current GRCr8 (GCF_036323735.1) 19 NC_086037.1 (68025619..68059754)
    RS_2023_06 previous assembly mRatBN7.2 (GCF_015227675.2) 19 NC_051354.1 (51117088..51151228)
    106 previous assembly Rnor_6.0 (GCF_000001895.5) 19 NC_005118.4 (55880549..55914729)

    Chromosome 19 - NC_086037.1Genomic Context describing neighboring genes Neighboring gene small nucleolar RNA U3 Neighboring gene uncharacterized LOC102551789 Neighboring gene ribosomal protein S13, pseudogene 1 Neighboring gene small nucleolar RNA MBII-202 Neighboring gene ribosomal protein L13 Neighboring gene copine 7

    Genomic regions, transcripts, and products

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    Go to nucleotide: Graphics FASTA GenBank

    Expression

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    • Project title: A rat RNA-Seq transcriptomic BodyMap across 11 organs and 4 developmental stages
    • Description: 320 RNA samples isolated from 11 organs (adrenal gland, brain, heart, kidney, liver, lung, muscle, spleen, thymus, and testes or uterus) from both sexes of Fischer 344 rats across four developmental stages (2-, 6-, 21-, and 104-weeks-old)
    • BioProject: PRJNA238328
    • Publication: PMID 24510058
    • Analysis date: Mon Jun 6 17:44:12 2016

    Bibliography

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    Related articles in PubMed

    1. Axonal degeneration in paraplegin-deficient mice is associated with abnormal mitochondria and impairment of axonal transport. Ferreirinha F, et al. J Clin Invest, 2004 Jan. PMID 14722615, Free PMC Article
    2. Molecular and functional analyses of the human and mouse genes encoding AFG3L1, a mitochondrial metalloprotease homologous to the human spastic paraplegia protein. Kremmidiotis G, et al. Genomics, 2001 Aug. PMID 11549317
    3. SPG7 Is an Essential and Conserved Component of the Mitochondrial Permeability Transition Pore. Shanmughapriya S, et al. Mol Cell, 2015 Oct 1. PMID 26387735, Free PMC Article
    4. Autocatalytic processing of m-AAA protease subunits in mitochondria. Koppen M, et al. Mol Biol Cell, 2009 Oct. PMID 19656850, Free PMC Article
    5. Integrated analysis of protein composition, tissue diversity, and gene regulation in mouse mitochondria. Mootha VK, et al. Cell, 2003 Nov 26. PMID 14651853

    See all (7) citations in PubMed

    GeneRIFs: Gene References Into Functions

    Pathways from PubChem

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    General gene information

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    Markers

    Homology

    Gene Ontology Provided by RGD

    Function Evidence Code Pubs
    enables ATP binding IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables ATP hydrolysis activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables ATP-dependent H2AZ histone chaperone activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables ATP-dependent H3-H4 histone complex chaperone activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables ATP-dependent peptidase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables DNA clamp loader activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables chromatin extrusion motor activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables cohesin loader activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables metalloendopeptidase activity IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    enables metalloendopeptidase activity IEA
    Inferred from Electronic Annotation
    more info
    		  
    enables metalloendopeptidase activity ISS
    Inferred from Sequence or Structural Similarity
    more info
    		  
    enables zinc ion binding IEA
    Inferred from Electronic Annotation
    more info
    		  
    Process Evidence Code Pubs
    involved_in anterograde axonal transport IEA
    Inferred from Electronic Annotation
    more info
    		  
    acts_upstream_of_or_within anterograde axonal transport ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in biological_process ND
    No biological Data available
    more info
    		  
    involved_in chromatin looping IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in chromatin remodeling IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in mitochondrial outer membrane permeabilization involved in programmed cell death IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in mitochondrial outer membrane permeabilization involved in programmed cell death IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    involved_in mitochondrial protein processing IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    acts_upstream_of_or_within mitochondrion organization ISO
    Inferred from Sequence Orthology
    more info
    		  
    involved_in proteolysis IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in regulation of calcium import into the mitochondrion IEA
    Inferred from Electronic Annotation
    more info
    		  
    involved_in regulation of calcium import into the mitochondrion ISS
    Inferred from Sequence or Structural Similarity
    more info
    		  
    involved_in regulation of mitochondrial membrane permeability IMP
    Inferred from Mutant Phenotype
    more info
    		 PubMed 
    Component Evidence Code Pubs
    located_in axon cytoplasm IEA
    Inferred from Electronic Annotation
    more info
    		  
    part_of m-AAA complex IBA
    Inferred from Biological aspect of Ancestor
    more info
    		  
    part_of m-AAA complex IEA
    Inferred from Electronic Annotation
    more info
    		  
    part_of m-AAA complex ISO
    Inferred from Sequence Orthology
    more info
    		  
    located_in membrane IEA
    Inferred from Electronic Annotation
    more info
    		  
    located_in mitochondrial inner membrane ISO
    Inferred from Sequence Orthology
    more info
    		  
    located_in mitochondrial inner membrane ISS
    Inferred from Sequence or Structural Similarity
    more info
    		  
    part_of mitochondrial permeability transition pore complex IEA
    Inferred from Electronic Annotation
    more info
    		  
    located_in mitochondrion ISO
    Inferred from Sequence Orthology
    more info
    		  

    General protein information

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    Preferred Names
    mitochondrial inner membrane m-AAA protease component paraplegin; paraplegin
    Names
    SPG7, paraplegin matrix AAA peptidase subunit
    spastic paraplegia 7 homolog

    NCBI Reference Sequences (RefSeq)

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    RefSeqs maintained independently of Annotated Genomes

    These reference sequences exist independently of genome builds. Explain

    These reference sequences are curated independently of the genome annotation cycle, so their versions may not match the RefSeq versions in the current genome build. Identify version mismatches by comparing the version of the RefSeq in this section to the one reported in Genomic regions, transcripts, and products above.

    mRNA and Protein(s)

    1. NM_001399110.1 → NP_001386039.1  mitochondrial inner membrane m-AAA protease component paraplegin isoform 2

      Status: VALIDATED

      Source sequence(s)
      JAXUCZ010000019
      UniProtKB/Swiss-Prot
      A0A0G2K536, Q7TT47
      UniProtKB/TrEMBL
      A0A8L2R710
      Related
      ENSRNOP00000073276.3, ENSRNOT00000091712.3
      Conserved Domains (2) summary
      TIGR01241
      Location:265 → 748
      FtsH_fam; ATP-dependent metalloprotease FtsH
      pfam06480
      Location:145 → 242
      FtsH_ext; FtsH Extracellular

    2. NM_181388.3 → NP_852053.1  mitochondrial inner membrane m-AAA protease component paraplegin isoform 1

      See identical proteins and their annotated locations for NP_852053.1

      Status: VALIDATED

      Source sequence(s)
      JAXUCZ010000019
      UniProtKB/TrEMBL
      A0A2H2GPM9, A0A8L2R710, A6IZU9
      Related
      ENSRNOP00000048848.2, ENSRNOT00000047754.5
      Conserved Domains (5) summary
      TIGR01241
      Location:265 → 711
      FtsH_fam; ATP-dependent metalloprotease FtsH
      pfam00004
      Location:346 → 479
      AAA; ATPase family associated with various cellular activities (AAA)
      pfam01434
      Location:524 → 709
      Peptidase_M41; Peptidase family M41
      pfam06480
      Location:145 → 242
      FtsH_ext; FtsH Extracellular
      cl21455
      Location:308 → 364
      P-loop_NTPase; P-loop containing Nucleoside Triphosphate Hydrolases

    RefSeqs of Annotated Genomes: GCF_036323735.1-RS_2024_02

    The following sections contain reference sequences that belong to a specific genome build. Explain

    Reference GRCr8

    Genomic

    1. NC_086037.1 Reference GRCr8

      Range
      68025619..68059754
      Download
      GenBank, FASTA, Sequence Viewer (Graphics)

    mRNA and Protein(s)

    1. XM_039097806.2 → XP_038953734.1  paraplegin isoform X1

      UniProtKB/TrEMBL
      A0A8L2R710
      Conserved Domains (2) summary
      TIGR01241
      Location:264 → 747
      FtsH_fam; ATP-dependent metalloprotease FtsH
      pfam06480
      Location:144 → 241
      FtsH_ext; FtsH Extracellular

    2. XM_063278101.1 → XP_063134171.1  paraplegin isoform X2

      UniProtKB/TrEMBL
      A0A8L2R710

    RNA

    1. XR_010060009.1 RNA Sequence

    2. XR_010060010.1 RNA Sequence

    Nucleotide Protein Strain
    Heading Accession and Version
    Protein Accession Links
    GenPept Link UniProtKB Link
    Q7TT47.2 GenPept UniProtKB/Swiss-Prot:Q7TT47

    Gene LinkOut

    The following LinkOut resources are supplied by external providers. These providers are responsible for maintaining the links.

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