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O96011 (PX11B_HUMAN) Reviewed, UniProtKB/Swiss-Prot

Last modified July 9, 2014. Version 105. Feed History...

Clusters with 100%, 90%, 50% identity | Documents (3) | Third-party data text xml rdf/xml gff fasta
to top of pageNames·Attributes·General annotation·Ontologies·Interactions·Alt products·Sequence annotation·Sequences·References·Cross-refs·Entry info·DocumentsCustomize order

Names and origin

Protein namesRecommended name:
Peroxisomal membrane protein 11B
Alternative name(s):
Peroxin-11B
Peroxisomal biogenesis factor 11B
Protein PEX11 homolog beta
Short name=PEX11-beta
Gene names
Name:PEX11B
OrganismHomo sapiens (Human) [Reference proteome]
Taxonomic identifier9606 [NCBI]
Taxonomic lineageEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo

Protein attributes

Sequence length259 AA.
Sequence statusComplete.
Protein existenceEvidence at protein level

General annotation (Comments)

Function

Involved in peroxisomal proliferation. May regulate peroxisomes division by recruiting the dynamin-related GTPase DNM1L to the peroxisomal membrane. Ref.2 Ref.9

Subunit structure

Interacts with PEX19. Ref.8

Subcellular location

Peroxisome membrane; Single-pass membrane protein Ref.1 Ref.2 Ref.8.

Involvement in disease

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.
Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.12

Sequence similarities

Belongs to the peroxin-11 family.

Caution

Ref.2 states that both the N- and the C-terminus are located in the cytoplasm.

Binary interactions

With

Entry

#Exp.

IntAct

Notes

PEX19P4085511EBI-594824,EBI-594747

Alternative products

This entry describes 2 isoforms produced by alternative splicing. [Align] [Select]
Isoform 1 (identifier: O96011-1)

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.
Isoform 2 (identifier: O96011-2)

The sequence of this isoform differs from the canonical sequence as follows:
     1-18: MDAWVRFSAQSQARERLC → MGKL
Note: No experimental confirmation available.

Sequence annotation (Features)

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifier

Molecule processing

Chain1 – 259259Peroxisomal membrane protein 11B
PRO_0000105967

Regions

Transmembrane233 – 25523Helical; Potential
Region211 – 25949Interaction with PEX19 and peroxisome targeting
Compositional bias159 – 18224Gly-rich

Amino acid modifications

Modified residue431N6-acetyllysine Ref.10

Natural variations

Alternative sequence1 – 1818MDAWV…RERLC → MGKL in isoform 2.
VSP_042860

Experimental info

Sequence conflict981A → V in AAH11963. Ref.7

Sequences

Sequence LengthMass (Da)Tools
Isoform 1 [UniParc].

Last modified May 1, 1999. Version 1.
Checksum: DC26CE30021C7B8D

FASTA25928,431
        10         20         30         40         50         60 
MDAWVRFSAQ SQARERLCRA AQYACSLLGH ALQRHGASPE LQKQIRQLES HLSLGRKLLR 

        70         80         90        100        110        120 
LGNSADALES AKRAVHLSDV VLRFCITVSH LNRALYFACD NVLWAGKSGL APRVDQEKWA 

       130        140        150        160        170        180 
QRSFRYYLFS LIMNLSRDAY EIRLLMEQES SACSRRLKGS GGGVPGGSET GGLGGPGTPG 

       190        200        210        220        230        240 
GGLPQLALKL RLQVLLLARV LRGHPPLLLD VVRNACDLFI PLDKLGLWRC GPGIVGLCGL 

       250 
VSSILSILTL IYPWLRLKP 

« Hide

Isoform 2 [UniParc].

Checksum: 48F9E9DDDA9F4447
Show »

FASTA24526,724

References

« Hide 'large scale' references
[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. expand/collapse author list , 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. expand/collapse author list , 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]Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M., Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J., Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S., Turner R. expand/collapse author list , Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H., Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K., Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D., Hunkapiller M.W., Myers E.W., Venter J.C.
Submitted (JUL-2005) to the EMBL/GenBank/DDBJ databases
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]"Initial characterization of the human central proteome."
Burkard T.R., Planyavsky M., Kaupe I., Breitwieser F.P., Buerckstuemmer T., Bennett K.L., Superti-Furga G., Colinge J.
BMC Syst. Biol. 5:17-17(2011) [PubMed] [Europe PMC] [Abstract]
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.
+Additional computationally mapped references.

Cross-references

Sequence databases

EMBL
GenBank
DDBJ
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.
CCDSCCDS53356.1. [O96011-2]
CCDS917.1. [O96011-1]
PIRJE0326.
RefSeqNP_001171724.1. NM_001184795.1. [O96011-2]
NP_003837.1. NM_003846.2. [O96011-1]
UniGeneHs.714608.

3D structure databases

ProteinModelPortalO96011.
ModBaseSearch...
MobiDBSearch...

Protein-protein interaction databases

BioGrid114327. 8 interactions.
IntActO96011. 3 interactions.
MINTMINT-241695.
STRING9606.ENSP00000358312.

PTM databases

PhosphoSiteO96011.

Proteomic databases

MaxQBO96011.
PaxDbO96011.
PeptideAtlasO96011.
PRIDEO96011.

Protocols and materials databases

DNASU8799.
StructuralBiologyKnowledgebaseSearch...

Genome annotation databases

EnsemblENST00000369306; ENSP00000358312; ENSG00000131779. [O96011-1]
ENST00000537888; ENSP00000437510; ENSG00000131779. [O96011-2]
ENST00000579989; ENSP00000464199; ENSG00000265832. [O96011-2]
ENST00000581144; ENSP00000463224; ENSG00000265832. [O96011-1]
GeneID8799.
KEGGhsa:8799.
UCSCuc001eny.2. human. [O96011-1]
uc010oyu.2. human. [O96011-2]

Organism-specific databases

CTD8799.
GeneCardsGC01P145516.
HGNCHGNC:8853. PEX11B.
HPAHPA050104.
MIM603867. gene.
614920. phenotype.
neXtProtNX_O96011.
Orphanet772. Infantile Refsum disease.
44. Neonatal adrenoleukodystrophy.
912. Zellweger syndrome.
PharmGKBPA33195.
GenAtlasSearch...

Phylogenomic databases

eggNOGNOG319767.
HOGENOMHOG000251589.
HOVERGENHBG053771.
InParanoidO96011.
KOK13352.
OMAHALQKHG.
PhylomeDBO96011.
TreeFamTF325704.

Gene expression databases

ArrayExpressO96011.
BgeeO96011.
CleanExHS_PEX11B.
GenevestigatorO96011.

Family and domain databases

InterProIPR008733. PEX11.
[Graphical view]
PfamPF05648. PEX11. 1 hit.
[Graphical view]
ProtoNetSearch...

Other

ChiTaRSPEX11B. human.
GeneWikiPEX11B.
GenomeRNAi8799.
NextBio33004.
PROO96011.
SOURCESearch...

Entry information

Entry namePX11B_HUMAN
AccessionPrimary (citable) accession number: O96011
Secondary accession number(s): B3KN85, B4DXH9, Q96ET2
Entry history
Integrated into UniProtKB/Swiss-Prot: April 26, 2005
Last sequence update: May 1, 1999
Last modified: July 9, 2014
This is version 105 of the entry and version 1 of the sequence. [Complete history]
Entry statusReviewed (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.

Relevant documents

SIMILARITY comments

Index of protein domains and families

MIM cross-references

Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot

Human chromosome 1

Human chromosome 1: entries, gene names and cross-references to MIM