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Q5XJY4

- PARL_MOUSE

UniProt

Q5XJY4 - PARL_MOUSE

Protein

Presenilins-associated rhomboid-like protein, mitochondrial

Gene

Parl

Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 96 (01 Oct 2014)
      Sequence version 1 (23 Nov 2004)
      Previous versions | rss
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    Functioni

    Required for the control of apoptosis during postnatal growth. Essential for proteolytic processing of an antiapoptotic form of OPA1 which prevents the release of mitochondrial cytochrome c in response to intrinsic apoptoptic signals By similarity.By similarity

    Catalytic activityi

    Cleaves type-1 transmembrane domains using a catalytic dyad composed of serine and histidine that are contributed by different transmembrane domains.

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Active sitei275 – 2751NucleophileBy similarity
    Active sitei333 – 3331By similarity

    GO - Molecular functioni

    1. serine-type endopeptidase activity Source: InterPro

    GO - Biological processi

    1. negative regulation of intrinsic apoptotic signaling pathway Source: UniProtKB
    2. negative regulation of release of cytochrome c from mitochondria Source: UniProtKB

    Keywords - Molecular functioni

    Hydrolase, Protease, Serine protease

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Presenilins-associated rhomboid-like protein, mitochondrial (EC:3.4.21.105)
    Alternative name(s):
    Mitochondrial intramembrane-cleaving protease PARL
    Cleaved into the following chain:
    P-beta
    Short name:
    Pbeta
    Gene namesi
    Name:Parl
    Synonyms:Psarl
    OrganismiMus musculus (Mouse)
    Taxonomic identifieri10090 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresGliresRodentiaSciurognathiMuroideaMuridaeMurinaeMusMus
    ProteomesiUP000000589: Chromosome 16

    Organism-specific databases

    MGIiMGI:1277152. Parl.

    Subcellular locationi

    Mitochondrion inner membrane 1 Publication; Multi-pass membrane protein 1 Publication
    Peptide P-beta : Nucleus
    Note: Translocated into the nucleus by an unknown mechanism.

    GO - Cellular componenti

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

    Keywords - Cellular componenti

    Membrane, Mitochondrion, Mitochondrion inner membrane, Nucleus

    Pathology & Biotechi

    Disruption phenotypei

    Mice develop normally until 4 weeks of age. They show subsequent progressive growth retardation, atrophy of muscle, spleen, and thymus as well as severe apoptosis of T and B lymphocytes leading to premature death between 8-12 weeks of age. Mouse embryonic fibroblasts lacking Parl show high susceptibility to intrinsic apoptotic signals. This defect can be complemented by Parl or a soluble form of Opa1.1 Publication

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transit peptidei1 – 5050MitochondrionBy similarityAdd
    BLAST
    Chaini51 – 377327Presenilins-associated rhomboid-like protein, mitochondrialPRO_0000027388Add
    BLAST
    Peptidei51 – 7525P-betaBy similarityPRO_0000027389Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei63 – 631PhosphoserineBy similarity
    Modified residuei68 – 681PhosphoserineBy similarity

    Post-translational modificationi

    P-beta is proteolytically processed (beta-cleavage) in a PARL-dependent manner.By similarity

    Keywords - PTMi

    Phosphoprotein

    Proteomic databases

    MaxQBiQ5XJY4.
    PaxDbiQ5XJY4.
    PRIDEiQ5XJY4.

    PTM databases

    PhosphoSiteiQ5XJY4.

    Expressioni

    Gene expression databases

    BgeeiQ5XJY4.
    CleanExiMM_PARL.
    GenevestigatoriQ5XJY4.

    Interactioni

    Subunit structurei

    Interacts with PSEN1 and PSEN2 By similarity. Binds OPA1.By similarity1 Publication

    Protein-protein interaction databases

    DIPiDIP-59825N.
    IntActiQ5XJY4. 1 interaction.
    STRINGi10090.ENSMUSP00000045361.

    Structurei

    3D structure databases

    ProteinModelPortaliQ5XJY4.
    ModBaseiSearch...
    MobiDBiSearch...

    Topological domain

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Topological domaini51 – 9949Mitochondrial matrixSequence AnalysisAdd
    BLAST
    Topological domaini120 – 16546Mitochondrial intermembraneSequence AnalysisAdd
    BLAST
    Topological domaini186 – 20520Mitochondrial matrixSequence AnalysisAdd
    BLAST
    Topological domaini229 – 24214Mitochondrial intermembraneSequence AnalysisAdd
    BLAST
    Topological domaini261 – 27010Mitochondrial matrixSequence Analysis
    Topological domaini288 – 2936Mitochondrial intermembraneSequence Analysis
    Topological domaini317 – 33014Mitochondrial matrixSequence AnalysisAdd
    BLAST
    Topological domaini353 – 37725Mitochondrial intermembraneSequence AnalysisAdd
    BLAST

    Transmembrane

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transmembranei100 – 11920HelicalSequence AnalysisAdd
    BLAST
    Transmembranei166 – 18520HelicalSequence AnalysisAdd
    BLAST
    Transmembranei206 – 22823HelicalSequence AnalysisAdd
    BLAST
    Transmembranei243 – 26018HelicalSequence AnalysisAdd
    BLAST
    Transmembranei271 – 28717HelicalSequence AnalysisAdd
    BLAST
    Transmembranei294 – 31623HelicalSequence AnalysisAdd
    BLAST
    Transmembranei331 – 35222HelicalSequence AnalysisAdd
    BLAST

    Family & Domainsi

    Sequence similaritiesi

    Belongs to the peptidase S54 family.Curated

    Keywords - Domaini

    Transit peptide, Transmembrane, Transmembrane helix

    Phylogenomic databases

    eggNOGiCOG0705.
    GeneTreeiENSGT00390000013063.
    HOGENOMiHOG000230670.
    HOVERGENiHBG082107.
    InParanoidiQ5XJY4.
    KOiK09650.
    OMAiHEMRTNS.
    OrthoDBiEOG7NGQBT.
    PhylomeDBiQ5XJY4.
    TreeFamiTF313603.

    Family and domain databases

    Gene3Di1.20.1540.10. 1 hit.
    InterProiIPR002610. Peptidase_S54_rhomboid.
    IPR022764. Peptidase_S54_rhomboid_dom.
    [Graphical view]
    PANTHERiPTHR22936. PTHR22936. 1 hit.
    PfamiPF01694. Rhomboid. 1 hit.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

    Sequence processingi: The displayed sequence is further processed into a mature form.

    Q5XJY4-1 [UniParc]FASTAAdd to Basket

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    MALQGWVQRG WRCGPAWAPP LGGGYRELSA TQAPRLLGRR FNLFVQQKCG    50
    FRKAPRKVEP RRSDTGSSGE AYKRSALIPP LEETVFYPSP YPIRTLVKPF 100
    FFTIGFTGCA FGSAAIWQYE SLKSRVQSYF DGIKADWLDS IRPQKEGNLR 150
    KEINKWWNSL SDGQRTVTGI IAANALVFCL WRVPSLQRTM IRYFTSNPAS 200
    KVLCSPMLLS TFSHFSLFHM AANMYVLWSF SSSIVNILGQ EQFVAVYLSA 250
    GVISNFVSYV CKVATGRYGP SLGASGAIMT VLAAVCTKIP EGRLAIIFLP 300
    VFTFTAGNAL KAIIAMDTAG MILGWKFFDH AAHLGGALFG IWYITYGHEL 350
    IWKNREPLVK IWHEIRTNGP KKGGGSK 377
    Length:377
    Mass (Da):41,964
    Last modified:November 23, 2004 - v1
    Checksum:i41C81310031A2EC4
    GO

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AK151652 mRNA. Translation: BAE30581.1.
    BC083153 mRNA. Translation: AAH83153.1.
    CCDSiCCDS28044.1.
    RefSeqiNP_001005767.1. NM_001005767.4.
    UniGeneiMm.371737.

    Genome annotation databases

    EnsembliENSMUST00000048642; ENSMUSP00000045361; ENSMUSG00000033918.
    GeneIDi381038.
    KEGGimmu:381038.
    UCSCiuc007ypm.1. mouse.

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AK151652 mRNA. Translation: BAE30581.1 .
    BC083153 mRNA. Translation: AAH83153.1 .
    CCDSi CCDS28044.1.
    RefSeqi NP_001005767.1. NM_001005767.4.
    UniGenei Mm.371737.

    3D structure databases

    ProteinModelPortali Q5XJY4.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    DIPi DIP-59825N.
    IntActi Q5XJY4. 1 interaction.
    STRINGi 10090.ENSMUSP00000045361.

    PTM databases

    PhosphoSitei Q5XJY4.

    Proteomic databases

    MaxQBi Q5XJY4.
    PaxDbi Q5XJY4.
    PRIDEi Q5XJY4.

    Protocols and materials databases

    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENSMUST00000048642 ; ENSMUSP00000045361 ; ENSMUSG00000033918 .
    GeneIDi 381038.
    KEGGi mmu:381038.
    UCSCi uc007ypm.1. mouse.

    Organism-specific databases

    CTDi 55486.
    MGIi MGI:1277152. Parl.

    Phylogenomic databases

    eggNOGi COG0705.
    GeneTreei ENSGT00390000013063.
    HOGENOMi HOG000230670.
    HOVERGENi HBG082107.
    InParanoidi Q5XJY4.
    KOi K09650.
    OMAi HEMRTNS.
    OrthoDBi EOG7NGQBT.
    PhylomeDBi Q5XJY4.
    TreeFami TF313603.

    Miscellaneous databases

    NextBioi 401496.
    PROi Q5XJY4.
    SOURCEi Search...

    Gene expression databases

    Bgeei Q5XJY4.
    CleanExi MM_PARL.
    Genevestigatori Q5XJY4.

    Family and domain databases

    Gene3Di 1.20.1540.10. 1 hit.
    InterProi IPR002610. Peptidase_S54_rhomboid.
    IPR022764. Peptidase_S54_rhomboid_dom.
    [Graphical view ]
    PANTHERi PTHR22936. PTHR22936. 1 hit.
    Pfami PF01694. Rhomboid. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "The transcriptional landscape of the mammalian genome."
      Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., Davis M.J.
      , Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., Hayashizaki Y.
      Science 309:1559-1563(2005) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Strain: C57BL/6J.
      Tissue: Bone marrow.
    2. "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].
      Tissue: Embryonic stem cell.
    3. "Mitochondrial rhomboid PARL regulates cytochrome c release during apoptosis via OPA1-dependent cristae remodeling."
      Cipolat S., Rudka T., Hartmann D., Costa V., Serneels L., Craessaerts K., Metzger K., Frezza C., Annaert W., D'Adamio L., Derks C., Dejaegere T., Pellegrini L., D'Hooge R., Scorrano L., De Strooper B.
      Cell 126:163-175(2006) [PubMed] [Europe PMC] [Abstract]
      Cited for: FUNCTION, SUBCELLULAR LOCATION, INTERACTION WITH OPA, DISRUPTION PHENOTYPE.

    Entry informationi

    Entry nameiPARL_MOUSE
    AccessioniPrimary (citable) accession number: Q5XJY4
    Secondary accession number(s): Q3U9T5, Q641T5
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: April 12, 2005
    Last sequence update: November 23, 2004
    Last modified: October 1, 2014
    This is version 96 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, 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