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Q5XJY4

- PARL_MOUSE

UniProt

Q5XJY4 - PARL_MOUSE

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Protein

Presenilins-associated rhomboid-like protein, mitochondrial

Gene
Parl, Psarl
Organism
Mus musculus (Mouse)
Status
Reviewed - Annotation score: 5 out of 5 - Experimental evidence at protein leveli

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.1 Publication

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 – 2751Nucleophile By similarity
Active sitei333 – 3331 By 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
Complete GO annotation...

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

Topology

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Topological domaini51 – 9949Mitochondrial matrix Reviewed predictionAdd
BLAST
Transmembranei100 – 11920Helical; Reviewed predictionAdd
BLAST
Topological domaini120 – 16546Mitochondrial intermembrane Reviewed predictionAdd
BLAST
Transmembranei166 – 18520Helical; Reviewed predictionAdd
BLAST
Topological domaini186 – 20520Mitochondrial matrix Reviewed predictionAdd
BLAST
Transmembranei206 – 22823Helical; Reviewed predictionAdd
BLAST
Topological domaini229 – 24214Mitochondrial intermembrane Reviewed predictionAdd
BLAST
Transmembranei243 – 26018Helical; Reviewed predictionAdd
BLAST
Topological domaini261 – 27010Mitochondrial matrix Reviewed prediction
Transmembranei271 – 28717Helical; Reviewed predictionAdd
BLAST
Topological domaini288 – 2936Mitochondrial intermembrane Reviewed prediction
Transmembranei294 – 31623Helical; Reviewed predictionAdd
BLAST
Topological domaini317 – 33014Mitochondrial matrix Reviewed predictionAdd
BLAST
Transmembranei331 – 35222Helical; Reviewed predictionAdd
BLAST
Topological domaini353 – 37725Mitochondrial intermembrane Reviewed predictionAdd
BLAST

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
Complete GO annotation...

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 – 5050Mitochondrion By similarityAdd
BLAST
Chaini51 – 377327Presenilins-associated rhomboid-like protein, mitochondrialPRO_0000027388Add
BLAST
Peptidei51 – 7525P-beta By similarityPRO_0000027389Add
BLAST

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei63 – 631Phosphoserine By similarity
Modified residuei68 – 681Phosphoserine By 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 OPA1.1 Publication

Protein-protein interaction databases

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

Structurei

3D structure databases

ProteinModelPortaliQ5XJY4.

Family & Domainsi

Sequence similaritiesi

Belongs to the peptidase S54 family.

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

« Hide

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

« Hide 'large scale' publications
  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: July 9, 2014
This is version 95 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

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