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P40855

- PEX19_HUMAN

UniProt

P40855 - PEX19_HUMAN

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Protein
Peroxisomal biogenesis factor 19
Gene
PEX19, HK33, PXF, OK/SW-cl.22
Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5 - Experimental evidence at protein leveli

Functioni

Necessary for early peroxisomal biogenesis. Acts both as a cytosolic chaperone and as an import receptor for peroxisomal membrane proteins (PMPs). Binds and stabilizes newly synthesized PMPs in the cytoplasm by interacting with their hydrophobic membrane-spanning domains, and targets them to the peroxisome membrane by binding to the integral membrane protein PEX3. Excludes CDKN2A from the nucleus and prevents its interaction with MDM2, which results in active degradation of TP53.6 Publications

GO - Molecular functioni

  1. ATPase binding Source: UniProtKB
  2. peroxisome membrane class-1 targeting sequence binding Source: UniProtKB
  3. protein N-terminus binding Source: UniProtKB
  4. protein binding Source: UniProtKB
Complete GO annotation...

GO - Biological processi

  1. chaperone-mediated protein folding Source: UniProtKB
  2. chaperone-mediated protein transport Source: UniProtKB
  3. establishment of protein localization to peroxisome Source: UniProtKB
  4. negative regulation of lipid binding Source: UniProtKB
  5. peroxisome fission Source: UniProtKB
  6. peroxisome membrane biogenesis Source: UniProtKB
  7. peroxisome organization Source: UniProtKB
  8. protein import into peroxisome membrane Source: UniProtKB
  9. protein stabilization Source: UniProtKB
  10. protein targeting to peroxisome Source: UniProtKB
  11. transmembrane transport Source: Reactome
Complete GO annotation...

Keywords - Biological processi

Peroxisome biogenesis

Enzyme and pathway databases

ReactomeiREACT_111158. ABCA transporters in lipid homeostasis.

Protein family/group databases

TCDBi9.A.17.1.2. the integral membrane peroxisomal protein importer-2 (ppi2) family.

Names & Taxonomyi

Protein namesi
Recommended name:
Peroxisomal biogenesis factor 19
Alternative name(s):
33 kDa housekeeping protein
Peroxin-19
Peroxisomal farnesylated protein
Gene namesi
Name:PEX19
Synonyms:HK33, PXF
ORF Names:OK/SW-cl.22
OrganismiHomo sapiens (Human)
Taxonomic identifieri9606 [NCBI]
Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
ProteomesiUP000005640: Chromosome 1

Organism-specific databases

HGNCiHGNC:9713. PEX19.

Subcellular locationi

Cytoplasm. Peroxisome membrane; Lipid-anchor; Cytoplasmic side
Note: Mainly cytoplasmic. Some fraction membrane-associated to the outer surface of peroxisomes.5 Publications

GO - Cellular componenti

  1. brush border membrane Source: UniProtKB
  2. cytoplasm Source: UniProtKB
  3. cytosol Source: UniProtKB
  4. integral component of membrane Source: UniProtKB
  5. intracellular membrane-bounded organelle Source: HPA
  6. nucleus Source: HPA
  7. peroxisomal membrane Source: UniProtKB
  8. peroxisome Source: UniProtKB
  9. protein complex Source: UniProtKB
Complete GO annotation...

Keywords - Cellular componenti

Cytoplasm, Membrane, Peroxisome

Pathology & Biotechi

Involvement in diseasei

Peroxisome biogenesis disorder complementation group 14 (PBD-CG14) [MIM:614886]: A peroxisomal disorder arising from a failure of protein import into the peroxisomal membrane or matrix. The peroxisome biogenesis disorders (PBD group) are genetically heterogeneous with at least 14 distinct genetic groups as concluded from complementation studies. Include disorders are: Zellweger syndrome (ZWS), neonatal adrenoleukodystrophy (NALD), infantile Refsum disease (IRD), and classical rhizomelic chondrodysplasia punctata (RCDP). ZWS, NALD and IRD are distinct from RCDP and constitute a clinical continuum of overlapping phenotypes known as the Zellweger spectrum (PBD-ZSS).
Note: The disease is caused by mutations affecting the gene represented in this entry.1 Publication
Peroxisome biogenesis disorder 12A (PBD12A) [MIM:614886]: A fatal peroxisome biogenesis disorder belonging to the Zellweger disease spectrum and clinically characterized by severe neurologic dysfunction with profound psychomotor retardation, severe hypotonia and neonatal seizures, craniofacial abnormalities, liver dysfunction, and biochemically by the absence of peroxisomes. Additional features include cardiovascular and skeletal defects, renal cysts, ocular abnormalities, and hearing impairment. Most severely affected individuals with the classic form of the disease (classic Zellweger syndrome) die within the first year of life.
Note: The disease is caused by mutations affecting the gene represented in this entry.1 Publication

Mutagenesis

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Mutagenesisi29 – 291F → A: Abolishes binding to PEX3. 1 Publication
Mutagenesisi296 – 2994Missing: Abolishes binding to PEX10, PEX11B, PEX12 and PEX13. Does not affect binding to PEX3 and PEX16. 5 Publications
Mutagenesisi296 – 2961C → A: Slightly inhibits PEX19 function on peroxisome biogenesis. 4 Publications
Mutagenesisi296 – 2961C → S: Abolishes farnesylation. Abolishes PEX19 function on peroxisome biogenesis. Does not affect binding to ABCD1, ABCD2 and ABCD3. 4 Publications

Keywords - Diseasei

Peroxisome biogenesis disorder, Zellweger syndrome

Organism-specific databases

MIMi614886. phenotype.
Orphaneti772. Infantile Refsum disease.
44. Neonatal adrenoleukodystrophy.
912. Zellweger syndrome.
PharmGKBiPA34058.

PTM / Processingi

Molecule processing

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Initiator methioninei1 – 11Removed1 Publication
Chaini2 – 296295Peroxisomal biogenesis factor 19
PRO_0000218759Add
BLAST
Propeptidei297 – 2993Removed in mature form Inferred
PRO_0000393944

Amino acid modifications

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Modified residuei2 – 21N-acetylalanine1 Publication
Modified residuei35 – 351Phosphoserine By similarity
Modified residuei296 – 2961Cysteine methyl ester Inferred
Lipidationi296 – 2961S-farnesyl cysteine2 Publications

Keywords - PTMi

Acetylation, Lipoprotein, Methylation, Phosphoprotein, Prenylation

Proteomic databases

MaxQBiP40855.
PaxDbiP40855.
PRIDEiP40855.

PTM databases

PhosphoSiteiP40855.

Expressioni

Tissue specificityi

Ubiquitously expressed. Isoform 1 is strongly predominant in all tissues except in utero where isoform 2 is the main form.1 Publication

Gene expression databases

ArrayExpressiP40855.
BgeeiP40855.
CleanExiHS_PEX19.
GenevestigatoriP40855.

Organism-specific databases

HPAiHPA044837.
HPA051966.

Interactioni

Subunit structurei

Interacts with a broad range of peroxisomal membrane proteins, including PEX3, PEX10, PEX11A, PEX11B, PEX12, PEX13, PEX14 and PEX16, PXMP2/PMP22, PXMP4/PMP24, SLC25A17/PMP34, ABCD1/ALDP, ABCD2/ALDRP, and ABCD3/PMP70. Also interacts with the tumor suppressor CDKN2A/p19ARF.9 Publications

Binary interactionsi

WithEntry#Exp.IntActNotes
ABCD1P338973EBI-594747,EBI-81045
ABCD3P282884EBI-594747,EBI-80992
PEX11BO9601111EBI-594747,EBI-594824
PEX12O006232EBI-594747,EBI-594836
PEX13Q9296812EBI-594747,EBI-594849
PEX14O7538115EBI-594747,EBI-594898
PEX16Q9Y5Y56EBI-594747,EBI-981985
PEX2P283283EBI-594747,EBI-713978
PEX26Q7Z4126EBI-594747,EBI-752057
PEX3P5658920EBI-594747,EBI-594885
PXMP2Q9NR772EBI-594747,EBI-1392944
SLC25A17O438084EBI-594747,EBI-594912

Protein-protein interaction databases

BioGridi111782. 37 interactions.
DIPiDIP-24172N.
IntActiP40855. 29 interactions.
MINTiMINT-241394.
STRINGi9606.ENSP00000357051.

Structurei

Secondary structure

Legend: HelixTurnBeta strand
Show more details
Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Helixi16 – 2712
Helixi28 – 314
Helixi67 – 7610
Helixi172 – 18211
Helixi185 – 19612
Helixi199 – 2068
Helixi207 – 2093
Helixi212 – 23423
Helixi241 – 26020
Helixi266 – 2683

3D structure databases

Select the link destinations:
PDBe
RCSB PDB
PDBj
Links Updated
EntryMethodResolution (Å)ChainPositionsPDBsum
2W85NMR-B66-77[»]
2WL8X-ray2.05A/B/C/D161-283[»]
3AJBX-ray2.50B1-44[»]
3MK4X-ray2.42B14-33[»]
ProteinModelPortaliP40855.
SMRiP40855. Positions 171-280.

Miscellaneous databases

EvolutionaryTraceiP40855.

Family & Domainsi

Region

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Regioni2 – 9190Necessary for PEX19 function on peroxisome biogenesis
Add
BLAST
Regioni2 – 5655Docking to the peroxisome membrane and binding to PEX3
Add
BLAST

Sequence similaritiesi

Belongs to the peroxin-19 family.

Phylogenomic databases

eggNOGiNOG133983.
HOGENOMiHOG000038537.
HOVERGENiHBG053573.
InParanoidiP40855.
KOiK13337.
OMAiNKAMEGL.
OrthoDBiEOG7P5T1P.
PhylomeDBiP40855.
TreeFamiTF315082.

Family and domain databases

InterProiIPR006708. Pex19.
[Graphical view]
PANTHERiPTHR12774. PTHR12774. 1 hit.
PfamiPF04614. Pex19. 1 hit.
[Graphical view]

Sequences (5)i

Sequence statusi: Complete.

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

This entry describes 5 isoformsi produced by alternative splicing. Align

Note: Experimental confirmation may be lacking for some isoforms.

Isoform 1 (identifier: P40855-1) [UniParc]FASTAAdd to Basket

Also known as: PXF-all

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

MAAAEEGCSV GAEADRELEE LLESALDDFD KAKPSPAPPS TTTAPDASGP    50
QKRSPGDTAK DALFASQEKF FQELFDSELA SQATAEFEKA MKELAEEEPH 100
LVEQFQKLSE AAGRVGSDMT SQQEFTSCLK ETLSGLAKNA TDLQNSSMSE 150
EELTKAMEGL GMDEGDGEGN ILPIMQSIMQ NLLSKDVLYP SLKEITEKYP 200
EWLQSHRESL PPEQFEKYQE QHSVMCKICE QFEAETPTDS ETTQKARFEM 250
VLDLMQQLQD LGHPPKELAG EMPPGLNFDL DALNLSGPPG ASGEQCLIM 299

Note: The two main transcripts are PXF-all and PXF-delta-2.

Length:299
Mass (Da):32,807
Last modified:February 1, 1995 - v1
Checksum:i399AF6B79F219100
GO
Isoform 2 (identifier: P40855-2)

Also known as: PXF-delta-2, PXF lacking exon 2

Sequence is not available

Note: The two main transcripts are PXF-all and PXF-delta-2.

Length:
Mass (Da):
Isoform 3 (identifier: P40855-3)

Also known as: PXF-delta-4, PXF lacking exon 4

Sequence is not available
Length:
Mass (Da):
Isoform 4 (identifier: P40855-4)

Also known as: PXF-delta-8, PXF lacking part of exon 8

Sequence is not available
Length:
Mass (Da):
Isoform 5 (identifier: P40855-5) [UniParc]FASTAAdd to Basket

The sequence of this isoform differs from the canonical sequence as follows:
     1-59: MAAAEEGCSV...PQKRSPGDTA → PPLRKAVVSGPKRTGNWRSFW

Note: Incomplete sequence.

Show »
Length:261
Mass (Da):29,259
Checksum:i5174D3C039E6A3B6
GO

Sequence cautioni

The sequence BAB93469.1 differs from that shown. Reason: Erroneous initiation.

Alternative sequence

Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
Alternative sequencei1 – 5959MAAAE…PGDTA → PPLRKAVVSGPKRTGNWRSF W in isoform 5.
VSP_012649Add
BLAST

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
X75535 mRNA. Translation: CAA53225.1.
Y09048 Genomic DNA. Translation: CAA70257.1.
AB018541 mRNA. Translation: BAA76291.1.
AB062286 mRNA. Translation: BAB93469.1. Different initiation.
BT006879 mRNA. Translation: AAP35525.1.
AL513282 Genomic DNA. Translation: CAI12457.1.
CH471121 Genomic DNA. Translation: EAW52728.1.
CH471121 Genomic DNA. Translation: EAW52729.1.
BC000496 mRNA. Translation: AAH00496.1.
CCDSiCCDS1201.1. [P40855-1]
PIRiI37468.
RefSeqiNP_001180573.1. NM_001193644.1.
NP_002848.1. NM_002857.3. [P40855-1]
UniGeneiHs.517232.

Genome annotation databases

EnsembliENST00000368072; ENSP00000357051; ENSG00000162735. [P40855-1]
GeneIDi5824.
KEGGihsa:5824.
UCSCiuc001fvs.2. human. [P40855-1]

Polymorphism databases

DMDMi729723.

Keywords - Coding sequence diversityi

Alternative splicing

Cross-referencesi

Sequence databases

Select the link destinations:
EMBL
GenBank
DDBJ
Links Updated
X75535 mRNA. Translation: CAA53225.1 .
Y09048 Genomic DNA. Translation: CAA70257.1 .
AB018541 mRNA. Translation: BAA76291.1 .
AB062286 mRNA. Translation: BAB93469.1 . Different initiation.
BT006879 mRNA. Translation: AAP35525.1 .
AL513282 Genomic DNA. Translation: CAI12457.1 .
CH471121 Genomic DNA. Translation: EAW52728.1 .
CH471121 Genomic DNA. Translation: EAW52729.1 .
BC000496 mRNA. Translation: AAH00496.1 .
CCDSi CCDS1201.1. [P40855-1 ]
PIRi I37468.
RefSeqi NP_001180573.1. NM_001193644.1.
NP_002848.1. NM_002857.3. [P40855-1 ]
UniGenei Hs.517232.

3D structure databases

Select the link destinations:
PDBe
RCSB PDB
PDBj
Links Updated
Entry Method Resolution (Å) Chain Positions PDBsum
2W85 NMR - B 66-77 [» ]
2WL8 X-ray 2.05 A/B/C/D 161-283 [» ]
3AJB X-ray 2.50 B 1-44 [» ]
3MK4 X-ray 2.42 B 14-33 [» ]
ProteinModelPortali P40855.
SMRi P40855. Positions 171-280.
ModBasei Search...

Protein-protein interaction databases

BioGridi 111782. 37 interactions.
DIPi DIP-24172N.
IntActi P40855. 29 interactions.
MINTi MINT-241394.
STRINGi 9606.ENSP00000357051.

Protein family/group databases

TCDBi 9.A.17.1.2. the integral membrane peroxisomal protein importer-2 (ppi2) family.

PTM databases

PhosphoSitei P40855.

Polymorphism databases

DMDMi 729723.

Proteomic databases

MaxQBi P40855.
PaxDbi P40855.
PRIDEi P40855.

Protocols and materials databases

DNASUi 5824.
Structural Biology Knowledgebase Search...

Genome annotation databases

Ensembli ENST00000368072 ; ENSP00000357051 ; ENSG00000162735 . [P40855-1 ]
GeneIDi 5824.
KEGGi hsa:5824.
UCSCi uc001fvs.2. human. [P40855-1 ]

Organism-specific databases

CTDi 5824.
GeneCardsi GC01M160246.
GeneReviewsi PEX19.
HGNCi HGNC:9713. PEX19.
HPAi HPA044837.
HPA051966.
MIMi 600279. gene.
614886. phenotype.
neXtProti NX_P40855.
Orphaneti 772. Infantile Refsum disease.
44. Neonatal adrenoleukodystrophy.
912. Zellweger syndrome.
PharmGKBi PA34058.
GenAtlasi Search...

Phylogenomic databases

eggNOGi NOG133983.
HOGENOMi HOG000038537.
HOVERGENi HBG053573.
InParanoidi P40855.
KOi K13337.
OMAi NKAMEGL.
OrthoDBi EOG7P5T1P.
PhylomeDBi P40855.
TreeFami TF315082.

Enzyme and pathway databases

Reactomei REACT_111158. ABCA transporters in lipid homeostasis.

Miscellaneous databases

EvolutionaryTracei P40855.
GeneWikii PEX19.
GenomeRNAii 5824.
NextBioi 22685.
PROi P40855.
SOURCEi Search...

Gene expression databases

ArrayExpressi P40855.
Bgeei P40855.
CleanExi HS_PEX19.
Genevestigatori P40855.

Family and domain databases

InterProi IPR006708. Pex19.
[Graphical view ]
PANTHERi PTHR12774. PTHR12774. 1 hit.
Pfami PF04614. Pex19. 1 hit.
[Graphical view ]
ProtoNeti Search...

Publicationsi

« Hide 'large scale' publications
  1. "Sequence of a putative human housekeeping gene (HK33) localized on chromosome 1."
    Braun A., Kammerer S., Weissenhorn W., Weiss E.H., Cleve H.
    Gene 146:291-295(1994) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1).
    Tissue: Ovary and Placenta.
  2. "Genomic organization and molecular characterization of a gene encoding HsPXF, a human peroxisomal farnesylated protein."
    Kammerer S., Arnold N., Gutensohn W., Mewes H.-W., Kunau W.-H., Hoefler G., Roscher A.A., Braun A.
    Genomics 45:200-210(1997) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [GENOMIC DNA], ALTERNATIVE SPLICING, TISSUE SPECIFICITY, SUBCELLULAR LOCATION, ISOPRENYLATION AT CYS-296, MUTAGENESIS OF CYS-296.
    Tissue: Leukocyte and Placenta.
  3. "Human PEX19: cDNA cloning by functional complementation, mutation analysis in a patient with Zellweger syndrome, and potential role in peroxisomal membrane assembly."
    Matsuzono Y., Kinoshita N., Tamura S., Shimozawa N., Hamasaki M., Ghaedi K., Wanders R.J.A., Suzuki Y., Kondo N., Fujiki Y.
    Proc. Natl. Acad. Sci. U.S.A. 96:2116-2121(1999) [PubMed] [Europe PMC] [Abstract]
    Cited for: NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 1), ISOPRENYLATION AT CYS-296, MUTAGENESIS OF CYS-296, SUBCELLULAR LOCATION, FUNCTION, INVOLVEMENT IN PBD12A.
    Tissue: Liver.
  4. "Identification of immuno-peptidmics that are recognized by tumor-reactive CTL generated from TIL of colon cancer patients."
    Shichijo S., Itoh K.
    Submitted (MAY-2001) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 5).
    Tissue: Colon adenocarcinoma.
  5. "Cloning of human full-length CDSs in BD Creator(TM) system donor vector."
    Kalnine N., Chen X., Rolfs A., Halleck A., Hines L., Eisenstein S., Koundinya M., Raphael J., Moreira D., Kelley T., LaBaer J., Lin Y., Phelan M., Farmer A.
    Submitted (MAY-2003) to the EMBL/GenBank/DDBJ databases
    Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
  6. "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].
  7. Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
  8. "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: Lung.
  9. "Human adrenoleukodystrophy protein and related peroxisomal ABC transporters interact with the peroxisomal assembly protein PEX19p."
    Gloeckner C.J., Mayerhofer P.U., Landgraf P., Muntau A.C., Holzinger A., Gerber J.-K., Kammerer S., Adamski J., Roscher A.A.
    Biochem. Biophys. Res. Commun. 271:144-150(2000) [PubMed] [Europe PMC] [Abstract]
    Cited for: INTERACTION WITH ABCD1; ABCD2 AND ABCD3, MUTAGENESIS OF CYS-296.
    Tissue: Brain.
  10. "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: FUNCTION, MUTAGENESIS OF CYS-296, SUBCELLULAR LOCATION, INTERACTION WITH ABCD1; ABCD2; ABCD3; PEX3; PEX10; PEX11A; PEX11B; PEX12; PEX13; PEX14; PEX16; PXMP2; PXMP4 AND SLC25A17.
  11. "Pex19p dampens the p19ARF-p53-p21WAF1 tumor suppressor pathway."
    Sugihara T., Kaul S.C., Kato J.-Y., Reddel R.R., Nomura H., Wadhwa R.
    J. Biol. Chem. 276:18649-18652(2001) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION, SUBCELLULAR LOCATION, INTERACTION WITH CDKN2A.
    Tissue: Testis.
  12. "Human pex19p binds peroxisomal integral membrane proteins at regions distinct from their sorting sequences."
    Fransen M., Wylin T., Brees C., Mannaerts G.P., Van Veldhoven P.P.
    Mol. Cell. Biol. 21:4413-4424(2001) [PubMed] [Europe PMC] [Abstract]
    Cited for: INTERACTION WITH PEX3; PEX10; PEX11B; PEX12; PEX13 AND PEX16, MUTAGENESIS OF 296-CYS--MET-299.
  13. "Two splice variants of human PEX19 exhibit distinct functions in peroxisomal assembly."
    Mayerhofer P.U., Kattenfeld T., Roscher A.A., Muntau A.C.
    Biochem. Biophys. Res. Commun. 291:1180-1186(2002) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION, INTERACTION WITH ABCD1; ABCD2; ABCD3 AND PEX3.
  14. "PEX3 functions as a PEX19 docking factor in the import of class I peroxisomal membrane proteins."
    Fang Y., Morrell J.C., Jones J.M., Gould S.J.
    J. Cell Biol. 164:863-875(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION, INTERACTION WITH PEX3, SUBCELLULAR LOCATION.
  15. "PEX19 is a predominantly cytosolic chaperone and import receptor for class 1 peroxisomal membrane proteins."
    Jones J.M., Morrell J.C., Gould S.J.
    J. Cell Biol. 164:57-67(2004) [PubMed] [Europe PMC] [Abstract]
    Cited for: FUNCTION, INTERACTION WITH PEX11B; PEX16; PXMP2; PXMP4; SLC25A17 AND ABCD3.
  16. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
    Tissue: Cervix carcinoma.
  17. "A mutation in PEX19 causes a severe clinical phenotype in a patient with peroxisomal biogenesis disorder."
    Mohamed S., El-Meleagy E., Nasr A., Ebberink M.S., Wanders R.J., Waterham H.R.
    Am. J. Med. Genet. A 152:2318-2321(2010) [PubMed] [Europe PMC] [Abstract]
    Cited for: INVOLVEMENT IN PBD-CG14.
  18. Cited for: IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
  19. Cited for: ACETYLATION [LARGE SCALE ANALYSIS] AT ALA-2, IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS], CLEAVAGE OF INITIATOR METHIONINE [LARGE SCALE ANALYSIS].
  20. "Structural basis for competitive interactions of Pex14 with the import receptors Pex5 and Pex19."
    Neufeld C., Filipp F.V., Simon B., Neuhaus A., Schueller N., David C., Kooshapur H., Madl T., Erdmann R., Schliebs W., Wilmanns M., Sattler M.
    EMBO J. 28:745-754(2009) [PubMed] [Europe PMC] [Abstract]
    Cited for: STRUCTURE BY NMR OF 66-77 IN COMPLEX WITH PEX14.
  21. "Structural basis for docking of peroxisomal membrane protein carrier Pex19p onto its receptor Pex3p."
    Sato Y., Shibata H., Nakatsu T., Nakano H., Kashiwayama Y., Imanaka T., Kato H.
    EMBO J. 29:4083-4093(2010) [PubMed] [Europe PMC] [Abstract]
    Cited for: X-RAY CRYSTALLOGRAPHY (2.50 ANGSTROMS) OF 1-44 IN COMPLEX WITH PEX3, SUBUNIT.
  22. "Insights into peroxisome function from the structure of PEX3 in complex with a soluble fragment of PEX19."
    Schmidt F., Treiber N., Zocher G., Bjelic S., Steinmetz M.O., Kalbacher H., Stehle T., Dodt G.
    J. Biol. Chem. 285:25410-25417(2010) [PubMed] [Europe PMC] [Abstract]
    Cited for: X-RAY CRYSTALLOGRAPHY (2.42 ANGSTROMS) OF 13-33 IN COMPLEX WITH PEX3, SUBUNIT, MUTAGENESIS OF PHE-29.

Entry informationi

Entry nameiPEX19_HUMAN
AccessioniPrimary (citable) accession number: P40855
Secondary accession number(s): D3DVE7, Q5QNY4, Q8NI97
Entry historyi
Integrated into UniProtKB/Swiss-Prot: February 1, 1995
Last sequence update: February 1, 1995
Last modified: September 3, 2014
This is version 148 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

3D-structure, 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. PDB cross-references
    Index of Protein Data Bank (PDB) cross-references
  4. SIMILARITY comments
    Index of protein domains and families

External Data

Dasty 3

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