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Q8JZQ2

- AFG32_MOUSE

UniProt

Q8JZQ2 - AFG32_MOUSE

Protein

AFG3-like protein 2

Gene

Afg3l2

Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 104 (01 Oct 2014)
      Sequence version 1 (01 Oct 2002)
      Previous versions | rss
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    Functioni

    ATP-dependent protease which is essential for axonal development.1 Publication

    Cofactori

    Binds 1 zinc ion per subunit.Curated

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Metal bindingi573 – 5731Zinc; catalyticBy similarity
    Active sitei574 – 5741By similarity
    Metal bindingi577 – 5771Zinc; catalyticBy similarity
    Metal bindingi648 – 6481Zinc; catalyticBy similarity

    Regions

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Nucleotide bindingi347 – 3548ATPSequence Analysis

    GO - Molecular functioni

    1. ATP binding Source: UniProtKB-KW
    2. metalloendopeptidase activity Source: InterPro
    3. nucleoside-triphosphatase activity Source: InterPro
    4. zinc ion binding Source: InterPro

    GO - Biological processi

    1. axonogenesis Source: MGI
    2. cristae formation Source: MGI
    3. death Source: MGI
    4. mitochondrial fusion Source: MGI
    5. mitochondrial protein processing Source: MGI
    6. mitochondrion organization Source: MGI
    7. muscle fiber development Source: MGI
    8. myelination Source: MGI
    9. nerve development Source: MGI
    10. neuromuscular junction development Source: MGI
    11. regulation of multicellular organism growth Source: MGI
    12. righting reflex Source: MGI

    Keywords - Molecular functioni

    Hydrolase, Metalloprotease, Protease

    Keywords - Ligandi

    ATP-binding, Metal-binding, Nucleotide-binding, Zinc

    Protein family/group databases

    MEROPSiM41.007.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    AFG3-like protein 2 (EC:3.4.24.-)
    Gene namesi
    Name:Afg3l2
    OrganismiMus musculus (Mouse)
    Taxonomic identifieri10090 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
    ProteomesiUP000000589: Chromosome 18

    Organism-specific databases

    MGIiMGI:1916847. Afg3l2.

    Subcellular locationi

    GO - Cellular componenti

    1. integral component of membrane Source: UniProtKB-KW
    2. mitochondrial inner membrane Source: MGI
    3. mitochondrion Source: MGI

    Keywords - Cellular componenti

    Membrane, Mitochondrion

    Pathology & Biotechi

    Involvement in diseasei

    Defects in Afg3l2 are the cause of the paralyze (par) phenotype, a spontaneous mutant strain. Par mice have a normal appearance and fertility but are significantly smaller than their littermates at 1 week of age and display a rapidly progressive loss of motor function in all limbs by 12-14 days. As the disease progresses, they lose the ability to support their own weight or turn themselves over when placed on their back and exhibit a typical posture with over extension of all limbs and uncoordinated movements. They rarely survive beyond 16 days of age, when they are completely paralyzed.

    Keywords - Diseasei

    Disease mutation

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini1 – 802802AFG3-like protein 2PRO_0000084674Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei116 – 1161N6-succinyllysine1 Publication

    Proteomic databases

    MaxQBiQ8JZQ2.
    PaxDbiQ8JZQ2.
    PRIDEiQ8JZQ2.

    PTM databases

    PhosphoSiteiQ8JZQ2.

    Expressioni

    Tissue specificityi

    Highly expressed in the cerebellar Purkinje cells.1 Publication

    Gene expression databases

    ArrayExpressiQ8JZQ2.
    BgeeiQ8JZQ2.
    CleanExiMM_AFG3L2.
    GenevestigatoriQ8JZQ2.

    Interactioni

    Subunit structurei

    Homooligomer. Interacts with SPG7; the interaction is required for the efficient assembly of mitochondrial complex I By similarity.By similarity

    Protein-protein interaction databases

    IntActiQ8JZQ2. 3 interactions.
    MINTiMINT-4116623.
    STRINGi10090.ENSMUSP00000025408.

    Structurei

    3D structure databases

    ProteinModelPortaliQ8JZQ2.
    SMRiQ8JZQ2. Positions 163-250, 295-749.
    ModBaseiSearch...
    MobiDBiSearch...

    Transmembrane

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transmembranei142 – 16221HelicalSequence AnalysisAdd
    BLAST
    Transmembranei250 – 27021HelicalSequence AnalysisAdd
    BLAST

    Family & Domainsi

    Sequence similaritiesi

    In the N-terminal section; belongs to the AAA ATPase family.Curated
    In the C-terminal section; belongs to the peptidase M41 family.Curated

    Keywords - Domaini

    Transmembrane, Transmembrane helix

    Phylogenomic databases

    eggNOGiCOG0465.
    GeneTreeiENSGT00730000110911.
    HOGENOMiHOG000217277.
    HOVERGENiHBG050184.
    InParanoidiQ8JZQ2.
    KOiK08956.
    OMAiMAHRCLR.
    OrthoDBiEOG7NW68F.
    PhylomeDBiQ8JZQ2.
    TreeFamiTF105004.

    Family and domain databases

    Gene3Di3.40.50.300. 1 hit.
    HAMAPiMF_01458. FtsH.
    InterProiIPR003593. AAA+_ATPase.
    IPR003959. ATPase_AAA_core.
    IPR003960. ATPase_AAA_CS.
    IPR005936. FtsH.
    IPR027417. P-loop_NTPase.
    IPR011546. Pept_M41_FtsH_extracell.
    IPR000642. Peptidase_M41.
    [Graphical view]
    PfamiPF00004. AAA. 1 hit.
    PF06480. FtsH_ext. 1 hit.
    PF01434. Peptidase_M41. 1 hit.
    [Graphical view]
    SMARTiSM00382. AAA. 1 hit.
    [Graphical view]
    SUPFAMiSSF52540. SSF52540. 1 hit.
    TIGRFAMsiTIGR01241. FtsH_fam. 1 hit.
    PROSITEiPS00674. AAA. 1 hit.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

    Q8JZQ2-1 [UniParc]FASTAAdd to Basket

    « Hide

    MAHRCLLLWS RGGCRRGLPP LLVPRGCLGP DRRPCLRTLY QYATVQTASS    50
    RRSLLRDVIA AYQRFCSRPP KGFEKYFPNG KNGKKASEPK EAVGEKKEPQ 100
    PSGPQPSGGA GGGGGKRRGK KEDSHWWSRF QKGDFPWDDK DFRMYFLWTA 150
    LFWGGVMIYF VFKSSGREIT WKDFVNNYLS KGVVDRLEVV NKRFVRVTFT 200
    PGKTPVDGQY VWFNIGSVDT FERNLETLQQ ELGIEGENRV PVVYIAESDG 250
    SFLLSMLPTV LIIAFLLYTI RRGPAGIGRT GRGMGGLFSV GETTAKVLKD 300
    EIDVKFKDVA GCEEAKLEIM EFVNFLKNPK QYQDLGAKIP KGAILTGPPG 350
    TGKTLLAKAT AGEANVPFIT VSGSEFLEMF VGVGPARVRD LFALARKNAP 400
    CILFIDEIDA VGRKRGRGNF GGQSEQENTL NQLLVEMDGF NTTTNVVILA 450
    GTNRPDILDP ALLRPGRFDR QIFIGPPDIK GRASIFKVHL RPLKLDSALE 500
    KDKLARKLAS LTPGFSGADV ANVCNEAALI AARHLSDAIN EKHFEQAIER 550
    VIGGLEKKTQ VLQPEEKKTV AYHEAGHAVA GWYLEHADPL LKVSIIPRGK 600
    GLGYAQYLPK EQYLYTKEQL LDRMCMTLGG RVSEEIFFGR ITTGAQDDLR 650
    KVTQSAYAQI VQFGMNEKVG QISFDLPRQG DMVLEKPYSE ATARMIDDEV 700
    RILISDAYRR TVALLTEKKA DVEKVALLLL EKEVLDKNDM VQLLGPRPFT 750
    EKSTYEEFVE GTGSLDEDTS LPEGLQDWNK EREKEEKKEK EKEEPLNEKV 800
    VS 802
    Length:802
    Mass (Da):89,519
    Last modified:October 1, 2002 - v1
    Checksum:iE7300BD686532D2D
    GO

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti389 – 3891R → G in par. 1 Publication

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    BC036999 mRNA. Translation: AAH36999.1.
    BC043056 mRNA. Translation: AAH43056.1.
    CCDSiCCDS37847.1.
    RefSeqiNP_081406.1. NM_027130.1.
    UniGeneiMm.426052.

    Genome annotation databases

    EnsembliENSMUST00000025408; ENSMUSP00000025408; ENSMUSG00000024527.
    GeneIDi69597.
    KEGGimmu:69597.
    UCSCiuc008fmf.1. mouse.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    BC036999 mRNA. Translation: AAH36999.1 .
    BC043056 mRNA. Translation: AAH43056.1 .
    CCDSi CCDS37847.1.
    RefSeqi NP_081406.1. NM_027130.1.
    UniGenei Mm.426052.

    3D structure databases

    ProteinModelPortali Q8JZQ2.
    SMRi Q8JZQ2. Positions 163-250, 295-749.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    IntActi Q8JZQ2. 3 interactions.
    MINTi MINT-4116623.
    STRINGi 10090.ENSMUSP00000025408.

    Protein family/group databases

    MEROPSi M41.007.

    PTM databases

    PhosphoSitei Q8JZQ2.

    Proteomic databases

    MaxQBi Q8JZQ2.
    PaxDbi Q8JZQ2.
    PRIDEi Q8JZQ2.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENSMUST00000025408 ; ENSMUSP00000025408 ; ENSMUSG00000024527 .
    GeneIDi 69597.
    KEGGi mmu:69597.
    UCSCi uc008fmf.1. mouse.

    Organism-specific databases

    CTDi 10939.
    MGIi MGI:1916847. Afg3l2.

    Phylogenomic databases

    eggNOGi COG0465.
    GeneTreei ENSGT00730000110911.
    HOGENOMi HOG000217277.
    HOVERGENi HBG050184.
    InParanoidi Q8JZQ2.
    KOi K08956.
    OMAi MAHRCLR.
    OrthoDBi EOG7NW68F.
    PhylomeDBi Q8JZQ2.
    TreeFami TF105004.

    Miscellaneous databases

    NextBioi 329880.
    PROi Q8JZQ2.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi Q8JZQ2.
    Bgeei Q8JZQ2.
    CleanExi MM_AFG3L2.
    Genevestigatori Q8JZQ2.

    Family and domain databases

    Gene3Di 3.40.50.300. 1 hit.
    HAMAPi MF_01458. FtsH.
    InterProi IPR003593. AAA+_ATPase.
    IPR003959. ATPase_AAA_core.
    IPR003960. ATPase_AAA_CS.
    IPR005936. FtsH.
    IPR027417. P-loop_NTPase.
    IPR011546. Pept_M41_FtsH_extracell.
    IPR000642. Peptidase_M41.
    [Graphical view ]
    Pfami PF00004. AAA. 1 hit.
    PF06480. FtsH_ext. 1 hit.
    PF01434. Peptidase_M41. 1 hit.
    [Graphical view ]
    SMARTi SM00382. AAA. 1 hit.
    [Graphical view ]
    SUPFAMi SSF52540. SSF52540. 1 hit.
    TIGRFAMsi TIGR01241. FtsH_fam. 1 hit.
    PROSITEi PS00674. AAA. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
      The MGC Project Team
      Genome Res. 14:2121-2127(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Strain: C57BL/6 and FVB/N.
      Tissue: Brain and Kidney.
    2. Lubec G., Kang S.U.
      Submitted (APR-2007) to UniProtKB
      Cited for: PROTEIN SEQUENCE OF 173-181; 283-296 AND 632-650, IDENTIFICATION BY MASS SPECTROMETRY.
      Strain: C57BL/6.
      Tissue: Brain.
    3. Cited for: FUNCTION, VARIANT PAR GLY-389.
    4. Cited for: TISSUE SPECIFICITY.
    5. "SIRT5-mediated lysine desuccinylation impacts diverse metabolic pathways."
      Park J., Chen Y., Tishkoff D.X., Peng C., Tan M., Dai L., Xie Z., Zhang Y., Zwaans B.M., Skinner M.E., Lombard D.B., Zhao Y.
      Mol. Cell 50:919-930(2013) [PubMed] [Europe PMC] [Abstract]
      Cited for: SUCCINYLATION [LARGE SCALE ANALYSIS] AT LYS-116, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
      Tissue: Liver.

    Entry informationi

    Entry nameiAFG32_MOUSE
    AccessioniPrimary (citable) accession number: Q8JZQ2
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: September 13, 2005
    Last sequence update: October 1, 2002
    Last modified: October 1, 2014
    This is version 104 of the entry and version 1 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Direct protein sequencing, Reference proteome

    Documents

    1. MGD cross-references
      Mouse Genome Database (MGD) cross-references in UniProtKB/Swiss-Prot
    2. Peptidase families
      Classification of peptidase families and list of entries
    3. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3