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O96011

- PX11B_HUMAN

UniProt

O96011 - PX11B_HUMAN

Protein

Peroxisomal membrane protein 11B

Gene

PEX11B

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 107 (01 Oct 2014)
      Sequence version 1 (01 May 1999)
      Previous versions | rss
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    Functioni

    Involved in peroxisomal proliferation. May regulate peroxisomes division by recruiting the dynamin-related GTPase DNM1L to the peroxisomal membrane.2 Publications

    GO - Molecular functioni

    1. protein binding Source: UniProtKB
    2. protein homodimerization activity Source: UniProtKB

    GO - Biological processi

    1. peroxisome fission Source: UniProtKB
    2. peroxisome organization Source: UniProtKB
    3. protein homooligomerization Source: UniProtKB
    4. regulation of peroxisome size Source: UniProtKB
    5. signal transduction Source: UniProtKB

    Keywords - Biological processi

    Peroxisome biogenesis

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Peroxisomal membrane protein 11B
    Alternative name(s):
    Peroxin-11B
    Peroxisomal biogenesis factor 11B
    Protein PEX11 homolog beta
    Short name:
    PEX11-beta
    Gene namesi
    Name:PEX11B
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 1

    Organism-specific databases

    HGNCiHGNC:8853. PEX11B.

    Subcellular locationi

    Peroxisome membrane 3 Publications; Single-pass membrane protein 3 Publications

    GO - Cellular componenti

    1. extracellular vesicular exosome Source: UniProt
    2. integral component of peroxisomal membrane Source: UniProtKB
    3. membrane Source: UniProtKB
    4. mitochondrion Source: Ensembl
    5. peroxisomal membrane Source: UniProtKB
    6. peroxisome Source: UniProtKB
    7. protein complex Source: UniProtKB

    Keywords - Cellular componenti

    Membrane, Peroxisome

    Pathology & Biotechi

    Involvement in diseasei

    Peroxisome biogenesis disorder 14B (PBD14B) [MIM:614920]: An autosomal recessive peroxisome biogenesis disorder characterized clinically by mild intellectual disability, congenital cataracts, progressive hearing loss, and polyneuropathy. Additionally, recurrent migraine-like episodes following mental stress or physical exertion, not a common feature in peroxisome disorders, are observed.1 Publication
    Note: The disease is caused by mutations affecting the gene represented in this entry.

    Keywords - Diseasei

    Peroxisome biogenesis disorder

    Organism-specific databases

    MIMi614920. phenotype.
    Orphaneti772. Infantile Refsum disease.
    44. Neonatal adrenoleukodystrophy.
    912. Zellweger syndrome.
    PharmGKBiPA33195.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Chaini1 – 259259Peroxisomal membrane protein 11BPRO_0000105967Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Modified residuei43 – 431N6-acetyllysine1 Publication

    Keywords - PTMi

    Acetylation

    Proteomic databases

    MaxQBiO96011.
    PaxDbiO96011.
    PeptideAtlasiO96011.
    PRIDEiO96011.

    PTM databases

    PhosphoSiteiO96011.

    Expressioni

    Gene expression databases

    ArrayExpressiO96011.
    BgeeiO96011.
    CleanExiHS_PEX11B.
    GenevestigatoriO96011.

    Organism-specific databases

    HPAiHPA050104.

    Interactioni

    Subunit structurei

    Interacts with PEX19.1 Publication

    Binary interactionsi

    WithEntry#Exp.IntActNotes
    PEX19P4085511EBI-594824,EBI-594747

    Protein-protein interaction databases

    BioGridi114327. 8 interactions.
    IntActiO96011. 3 interactions.
    MINTiMINT-241695.
    STRINGi9606.ENSP00000358312.

    Structurei

    3D structure databases

    ProteinModelPortaliO96011.
    ModBaseiSearch...
    MobiDBiSearch...

    Transmembrane

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Transmembranei233 – 25523HelicalSequence AnalysisAdd
    BLAST

    Family & Domainsi

    Region

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Regioni211 – 25949Interaction with PEX19 and peroxisome targetingAdd
    BLAST

    Compositional bias

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Compositional biasi159 – 18224Gly-richAdd
    BLAST

    Sequence similaritiesi

    Belongs to the peroxin-11 family.Curated

    Keywords - Domaini

    Transmembrane, Transmembrane helix

    Phylogenomic databases

    eggNOGiNOG319767.
    HOGENOMiHOG000251589.
    HOVERGENiHBG053771.
    InParanoidiO96011.
    KOiK13352.
    OMAiHALQKHG.
    PhylomeDBiO96011.
    TreeFamiTF325704.

    Family and domain databases

    InterProiIPR008733. PEX11.
    [Graphical view]
    PfamiPF05648. PEX11. 1 hit.
    [Graphical view]

    Sequences (2)i

    Sequence statusi: Complete.

    This entry describes 2 isoformsi produced by alternative splicing. Align

    Isoform 1 (identifier: O96011-1) [UniParc]FASTAAdd to Basket

    This isoform has been chosen as the 'canonical' sequence. All positional information in this entry refers to it. This is also the sequence that appears in the downloadable versions of the entry.

    « Hide

    MDAWVRFSAQ SQARERLCRA AQYACSLLGH ALQRHGASPE LQKQIRQLES    50
    HLSLGRKLLR LGNSADALES AKRAVHLSDV VLRFCITVSH LNRALYFACD 100
    NVLWAGKSGL APRVDQEKWA QRSFRYYLFS LIMNLSRDAY EIRLLMEQES 150
    SACSRRLKGS GGGVPGGSET GGLGGPGTPG GGLPQLALKL RLQVLLLARV 200
    LRGHPPLLLD VVRNACDLFI PLDKLGLWRC GPGIVGLCGL VSSILSILTL 250
    IYPWLRLKP 259
    Length:259
    Mass (Da):28,431
    Last modified:May 1, 1999 - v1
    Checksum:iDC26CE30021C7B8D
    GO
    Isoform 2 (identifier: O96011-2) [UniParc]FASTAAdd to Basket

    The sequence of this isoform differs from the canonical sequence as follows:
         1-18: MDAWVRFSAQSQARERLC → MGKL

    Note: No experimental confirmation available.

    Show »
    Length:245
    Mass (Da):26,724
    Checksum:i48F9E9DDDA9F4447
    GO

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti98 – 981A → V in AAH11963. (PubMed:15489334)Curated

    Alternative sequence

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Alternative sequencei1 – 1818MDAWV…RERLC → MGKL in isoform 2. 1 PublicationVSP_042860Add
    BLAST

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AB018080 mRNA. Translation: BAA34812.1.
    AF093670 mRNA. Translation: AAC78660.1.
    CR542047 mRNA. Translation: CAG46844.1.
    AK023991 mRNA. Translation: BAG51247.1.
    AK301983 mRNA. Translation: BAG63391.1.
    AL160282 Genomic DNA. No translation available.
    CH471244 Genomic DNA. Translation: EAW71422.1.
    BC011963 mRNA. Translation: AAH11963.1.
    CCDSiCCDS53356.1. [O96011-2]
    CCDS917.1. [O96011-1]
    PIRiJE0326.
    RefSeqiNP_001171724.1. NM_001184795.1. [O96011-2]
    NP_003837.1. NM_003846.2. [O96011-1]
    UniGeneiHs.714608.

    Genome annotation databases

    EnsembliENST00000369306; ENSP00000358312; ENSG00000131779. [O96011-1]
    ENST00000537888; ENSP00000437510; ENSG00000131779. [O96011-2]
    GeneIDi8799.
    KEGGihsa:8799.
    UCSCiuc001eny.2. human. [O96011-1]
    uc010oyu.2. human. [O96011-2]

    Keywords - Coding sequence diversityi

    Alternative splicing

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AB018080 mRNA. Translation: BAA34812.1 .
    AF093670 mRNA. Translation: AAC78660.1 .
    CR542047 mRNA. Translation: CAG46844.1 .
    AK023991 mRNA. Translation: BAG51247.1 .
    AK301983 mRNA. Translation: BAG63391.1 .
    AL160282 Genomic DNA. No translation available.
    CH471244 Genomic DNA. Translation: EAW71422.1 .
    BC011963 mRNA. Translation: AAH11963.1 .
    CCDSi CCDS53356.1. [O96011-2 ]
    CCDS917.1. [O96011-1 ]
    PIRi JE0326.
    RefSeqi NP_001171724.1. NM_001184795.1. [O96011-2 ]
    NP_003837.1. NM_003846.2. [O96011-1 ]
    UniGenei Hs.714608.

    3D structure databases

    ProteinModelPortali O96011.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 114327. 8 interactions.
    IntActi O96011. 3 interactions.
    MINTi MINT-241695.
    STRINGi 9606.ENSP00000358312.

    PTM databases

    PhosphoSitei O96011.

    Proteomic databases

    MaxQBi O96011.
    PaxDbi O96011.
    PeptideAtlasi O96011.
    PRIDEi O96011.

    Protocols and materials databases

    DNASUi 8799.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000369306 ; ENSP00000358312 ; ENSG00000131779 . [O96011-1 ]
    ENST00000537888 ; ENSP00000437510 ; ENSG00000131779 . [O96011-2 ]
    GeneIDi 8799.
    KEGGi hsa:8799.
    UCSCi uc001eny.2. human. [O96011-1 ]
    uc010oyu.2. human. [O96011-2 ]

    Organism-specific databases

    CTDi 8799.
    GeneCardsi GC01P145516.
    HGNCi HGNC:8853. PEX11B.
    HPAi HPA050104.
    MIMi 603867. gene.
    614920. phenotype.
    neXtProti NX_O96011.
    Orphaneti 772. Infantile Refsum disease.
    44. Neonatal adrenoleukodystrophy.
    912. Zellweger syndrome.
    PharmGKBi PA33195.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi NOG319767.
    HOGENOMi HOG000251589.
    HOVERGENi HBG053771.
    InParanoidi O96011.
    KOi K13352.
    OMAi HALQKHG.
    PhylomeDBi O96011.
    TreeFami TF325704.

    Miscellaneous databases

    ChiTaRSi PEX11B. human.
    GeneWikii PEX11B.
    GenomeRNAii 8799.
    NextBioi 33004.
    PROi O96011.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi O96011.
    Bgeei O96011.
    CleanExi HS_PEX11B.
    Genevestigatori O96011.

    Family and domain databases

    InterProi IPR008733. PEX11.
    [Graphical view ]
    Pfami PF05648. PEX11. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "cDNA cloning and characterization of a constitutively expressed isoform of the human peroxin Pex11p."
      Abe I., Fujiki Y.
      Biochem. Biophys. Res. Commun. 252:529-533(1998) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), SUBCELLULAR LOCATION.
      Tissue: Liver.
    2. "Expression of PEX11beta mediates peroxisome proliferation in the absence of extracellular stimuli."
      Schrader M., Reuber B.E., Morrell J.C., Jimenez-Sanchez G., Obie C., Stroh T.A., Valle D., Schroer T.A., Gould S.J.
      J. Biol. Chem. 273:29607-29614(1998) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), SUBCELLULAR LOCATION, FUNCTION.
    3. "Cloning of human full open reading frames in Gateway(TM) system entry vector (pDONR201)."
      Halleck A., Ebert L., Mkoundinya M., Schick M., Eisenstein S., Neubert P., Kstrang K., Schatten R., Shen B., Henze S., Mar W., Korn B., Zuo D., Hu Y., LaBaer J.
      Submitted (JUN-2004) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
    4. "Complete sequencing and characterization of 21,243 full-length human cDNAs."
      Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R., Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H., Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.
      , Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K., Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H., Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M., Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K., Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T., Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M., Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S., Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H., Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K., Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N., Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S., Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O., Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H., Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B., Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y., Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K., Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T., Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T., Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y., Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H., Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y., Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H., Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O., Isogai T., Sugano S.
      Nat. Genet. 36:40-45(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2).
      Tissue: Testis.
    5. "The DNA sequence and biological annotation of human chromosome 1."
      Gregory S.G., Barlow K.F., McLay K.E., Kaul R., Swarbreck D., Dunham A., Scott C.E., Howe K.L., Woodfine K., Spencer C.C.A., Jones M.C., Gillson C., Searle S., Zhou Y., Kokocinski F., McDonald L., Evans R., Phillips K.
      , Atkinson A., Cooper R., Jones C., Hall R.E., Andrews T.D., Lloyd C., Ainscough R., Almeida J.P., Ambrose K.D., Anderson F., Andrew R.W., Ashwell R.I.S., Aubin K., Babbage A.K., Bagguley C.L., Bailey J., Beasley H., Bethel G., Bird C.P., Bray-Allen S., Brown J.Y., Brown A.J., Buckley D., Burton J., Bye J., Carder C., Chapman J.C., Clark S.Y., Clarke G., Clee C., Cobley V., Collier R.E., Corby N., Coville G.J., Davies J., Deadman R., Dunn M., Earthrowl M., Ellington A.G., Errington H., Frankish A., Frankland J., French L., Garner P., Garnett J., Gay L., Ghori M.R.J., Gibson R., Gilby L.M., Gillett W., Glithero R.J., Grafham D.V., Griffiths C., Griffiths-Jones S., Grocock R., Hammond S., Harrison E.S.I., Hart E., Haugen E., Heath P.D., Holmes S., Holt K., Howden P.J., Hunt A.R., Hunt S.E., Hunter G., Isherwood J., James R., Johnson C., Johnson D., Joy A., Kay M., Kershaw J.K., Kibukawa M., Kimberley A.M., King A., Knights A.J., Lad H., Laird G., Lawlor S., Leongamornlert D.A., Lloyd D.M., Loveland J., Lovell J., Lush M.J., Lyne R., Martin S., Mashreghi-Mohammadi M., Matthews L., Matthews N.S.W., McLaren S., Milne S., Mistry S., Moore M.J.F., Nickerson T., O'Dell C.N., Oliver K., Palmeiri A., Palmer S.A., Parker A., Patel D., Pearce A.V., Peck A.I., Pelan S., Phelps K., Phillimore B.J., Plumb R., Rajan J., Raymond C., Rouse G., Saenphimmachak C., Sehra H.K., Sheridan E., Shownkeen R., Sims S., Skuce C.D., Smith M., Steward C., Subramanian S., Sycamore N., Tracey A., Tromans A., Van Helmond Z., Wall M., Wallis J.M., White S., Whitehead S.L., Wilkinson J.E., Willey D.L., Williams H., Wilming L., Wray P.W., Wu Z., Coulson A., Vaudin M., Sulston J.E., Durbin R.M., Hubbard T., Wooster R., Dunham I., Carter N.P., McVean G., Ross M.T., Harrow J., Olson M.V., Beck S., Rogers J., Bentley D.R.
      Nature 441:315-321(2006) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    6. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    7. "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] (ISOFORM 1).
      Tissue: Eye.
    8. "PEX19 binds multiple peroxisomal membrane proteins, is predominantly cytoplasmic, and is required for peroxisome membrane synthesis."
      Sacksteder K.A., Jones J.M., South S.T., Li X., Liu Y., Gould S.J.
      J. Cell Biol. 148:931-944(2000) [PubMed] [Europe PMC] [Abstract]
      Cited for: INTERACTION WITH PEX19, SUBCELLULAR LOCATION.
    9. "The dynamin-like GTPase DLP1 is essential for peroxisome division and is recruited to peroxisomes in part by PEX11."
      Li X., Gould S.J.
      J. Biol. Chem. 278:17012-17020(2003) [PubMed] [Europe PMC] [Abstract]
      Cited for: FUNCTION.
    10. "Lysine acetylation targets protein complexes and co-regulates major cellular functions."
      Choudhary C., Kumar C., Gnad F., Nielsen M.L., Rehman M., Walther T.C., Olsen J.V., Mann M.
      Science 325:834-840(2009) [PubMed] [Europe PMC] [Abstract]
      Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT LYS-43, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    11. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    12. "A novel defect of peroxisome division due to a homozygous non-sense mutation in the PEX11beta gene."
      Ebberink M.S., Koster J., Visser G., van Spronsen F., Stolte-Dijkstra I., Smit G.P., Fock J.M., Kemp S., Wanders R.J., Waterham H.R.
      J. Med. Genet. 49:307-313(2012) [PubMed] [Europe PMC] [Abstract]
      Cited for: INVOLVEMENT IN PBD14B.

    Entry informationi

    Entry nameiPX11B_HUMAN
    AccessioniPrimary (citable) accession number: O96011
    Secondary accession number(s): B3KN85, B4DXH9, Q96ET2
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: April 26, 2005
    Last sequence update: May 1, 1999
    Last modified: October 1, 2014
    This is version 107 of the entry and version 1 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program
    DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

    Miscellaneousi

    Keywords - Technical termi

    Complete proteome, Reference proteome

    Documents

    1. Human chromosome 1
      Human chromosome 1: entries, gene names and cross-references to MIM
    2. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    3. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3