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FLASH GENE
Symbol PEX19 contributors: mct/shn - updated : 29-01-2018
HGNC name peroxisomal biogenesis factor 19
HGNC id 9713
Corresponding disease
PBD11 peroxisome biogenesis disorder, Zellweger spectrum
ZWS5 Zellweger cerebrohepatorenal syndrome, variant type 5
Location 1q23.2      Physical location : 160.246.598 - 160.254.941
Synonym name
  • peroxin 19/peroxisome biogenesis factor 19
  • housekeeping gene 33kD
  • peroxisomal farnesylated protein
  • 33 kDa housekeeping protein
  • housekeeping gene, 33kD
    • Synonym symbol(s) HGNC9713, HSPXF, D1S2223E, HK33, PXF, PMP1, PMPI, PXMP1, OK/SW-cl.22
    DNA
    TYPE functioning gene
    STRUCTURE 8.34 kb     8 Exon(s)
    MAPPING cloned Y linked N status confirmed
    Map cen - D1S1167 - D1S2707 - PEX19 - D1S2771 - D1S2705 - qter
    RNA
    TRANSCRIPTS type messenger
    text
  • a functional diversity of the proteins encoded by two of four PEX19 splice variants (PMID: 11883941)
    • identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    8 splicing 3722 - 299 - 2016 27295553
    8 splicing 3642 - 279 - 2016 27295553
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart   highly
    Nervousbrain   highly
    Reproductivemale systemtestis  highly
    Respiratoryrespiratory tracttrachea  highly
    Visualeye    
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES Hydrophilic
    STRUCTURE
    motifs/domains
  • a N-terminusthat initiates the binding to PEX3, and then subtle conformational changes in PEX3 affect the surface of the PEX3 molecule
  • a conserved C-terminal farnesylation site
  • prenylation motif
  • a globular domain that displays a bundle of three long helices
  • a PMP-targeting signal (mPTS) at the C-terminal
  • C-terminal CAAX motif, and C-terminal region of PEX19 was not essential for the association of PEX19 with peroxisomal membrane and interaction with membrane assembly factors, PEX3 and PEX16
    • HOMOLOGY
    interspecies ortholog to Pex19, Rattus norvegicus
    ortholog to PEX19, Pan troglodytes
    ortholog to Pex19, Mus musculus
    ortholog to pex19, Danio rerio
    Homologene
    FAMILY
  • peroxin-19 family
    • CATEGORY chaperone/stress , receptor membrane
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,peroxisome
    intracellular,cytoplasm,cytosolic
    intracellular,nucleus,nucleoplasm
    text
  • attached to the outer surface of peroxisomes
  • associated with theendoplasmic reticulum
  • PEX19 was partly localized to peroxisomes but mostly localized in the cytosol
    • basic FUNCTION
  • involved in the process of peroxisomal biogenesis or assembly
  • a central role in the early steps of peroxisomal membrane synthesis
  • required for the import of peroxisome membrane proteins (class 1 exclusively) from cytoplasm to peroxisome
  • involved in the intracellular sorting and trafficking of ALDP, PMP70, and ALDRP proteins
  • might function as a PMP-specific chaperone, a cycling peroxisomal membrane proteins(PMP)-receptor protein, a PMP membrane insertion factor, or an association/dissociation factor of membrane-associated protein complexes
  • binds to the docking and assembly complex Pex13p/Pex14p, indicating a functional role in the formation of a multi-component importomer complex
  • farnesylation of PEX19 is required for its structural integrity and function in peroxisome biogenesis
  • functions as a receptor and chaperone of peroxisomal membrane proteins
  • PEX3 and PEX19 are essential for peroxisome biogenesis
  • PEX19 formed a complex with the peroxisomal Tail-anchored (TA) protein PEX26 in the cytosol and translocated it directly to peroxisomes by interacting with the peroxisomal membrane protein PEX3
  • in peroxisomes, peroxins PEX3 and PEX19 are the principal protein components of the machinery required for early peroxisomal biogenesis
  • transport of peroxisomal membrane proteins (PMPs) requires the soluble PEX19 protein as chaperone and import receptor
  • harbouring a prenylation CAAX box functions as a chaperone and transporter for peroxisomal membrane proteins in membrane assembly
  • PEX19 and PEX3 play an indispensable role in peroxisomal membrane protein (PMP) biogenesis, peroxisome division, and inheritance
  • PEX3, an integral peroxisomal membrane protein, and the cytosolic shuttling receptor PEX19 are thought to be responsible for the early steps of peroxisome biogenesis and assembly of their matrix protein import machinery
    • CELLULAR PROCESS cell organization/biogenesis
    PHYSIOLOGICAL PROCESS
    text early peroxisomal biogenesis
    PATHWAY
    metabolism
    signaling
    a component
  • crucial function of the PEX3-PEX19 complex during formation of peroxisomes in peroxisome-deficient cells, pointing to a dual function of the PEX3-PEX19 interaction in peroxisome biogenesis
    • INTERACTION
    DNA
    RNA
    small molecule
    protein
  • PEX12, PEX13, PEX3, PEX14, PMP70, PMP34
  • adrenoleukodystrophy protein, ALDP; adrenoleukodystrophy-related protein, ALDRP and Peroxisomal membrane protein-1 (70kD), PMP70
  • Pex16p
  • Na/P(i) IIa co-transporter
  • targeting of FIS1 to peroxisomes and mitochondria are independent events and support a direct, PEX19-dependent targeting of peroxisomal tail-anchored proteins (
  • PEX14 binds the soluble receptors PEX5 and PEX19
  • membrane-anchored PEX3 serves as the receptor for cytosolic PEX19, which in turn recognizes newly synthesized peroxisomal membrane proteins
  • interaction between PEX3, which resides on the peroxisomal membrane, and PEX19, which resides in the cytosol, is crucial for peroxisome formation and the post-translational targeting of peroxisomal membrane proteins (PMPs)
  • PEX19 is a cytosolic receptor harboring a C-terminal CAAX motif, which is responsible for the targeting of FAR1 to peroxisomes
  • within the cell, PEX3 is stabilized by PEX19 preventing PEX3 aggregation
  • peroxisomal targeting of ATAD1, an N-terminally signal-anchored protein that resides on both mitochondria and peroxisomes, is also achieved through the PEX19- and PEX3-dependent class I pathway 2)
  • PEX19 bridged the interaction at the ER between PEX3 and RING-domain proteins, resulting in a ternary complex that was critical for the intra-ER sorting and subsequent budding of the RING-domain peroxins
    • cell & other
  • attached to the outer surface of peroxisome
    • REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) ZWS5 , PBD11
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS