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O96011

- PX11B_HUMAN

UniProt

O96011 - PX11B_HUMAN

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Protein

Peroxisomal membrane protein 11B

Gene

PEX11B

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli

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 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
Complete GO annotation...

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

Topology

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

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
Complete GO annotation...

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

BgeeiO96011.
CleanExiHS_PEX11B.
ExpressionAtlasiO96011. baseline and differential.
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...

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.
GeneTreeiENSGT00390000014273.
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

        10         20         30         40         50
MDAWVRFSAQ SQARERLCRA AQYACSLLGH ALQRHGASPE LQKQIRQLES
60 70 80 90 100
HLSLGRKLLR LGNSADALES AKRAVHLSDV VLRFCITVSH LNRALYFACD
110 120 130 140 150
NVLWAGKSGL APRVDQEKWA QRSFRYYLFS LIMNLSRDAY EIRLLMEQES
160 170 180 190 200
SACSRRLKGS GGGVPGGSET GGLGGPGTPG GGLPQLALKL RLQVLLLARV
210 220 230 240 250
LRGHPPLLLD VVRNACDLFI PLDKLGLWRC GPGIVGLCGL VSSILSILTL

IYPWLRLKP
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:
EMBLi
GenBanki
DDBJi
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.
CCDSiCCDS72870.1. [O96011-2]
CCDS72871.1. [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:
EMBLi
GenBanki
DDBJi
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 CCDS72870.1. [O96011-2 ]
CCDS72871.1. [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.
GeneTreei ENSGT00390000014273.
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

Bgeei O96011.
CleanExi HS_PEX11B.
ExpressionAtlasi O96011. baseline and differential.
Genevestigatori O96011.

Family and domain databases

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

Publicationsi

« Hide 'large scale' publications
  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 29, 2014
This is version 108 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