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FLASH GENE
Symbol PEX11B contributors: mct - updated : 22-02-2016
HGNC name peroxisomal bigenesis factor 11 beta
HGNC id 8853
Corresponding disease
PBD13 peroxisome biogenesis disorder 13, Zellweger spectrum
Location 1q21.1      Physical location : 145.516.164 - 145.523.731
Synonym name
  • peroxisomal biogenesis factor 11B
  • peroxin-11B
  • protein PEX11 homolog beta
    • Synonym symbol(s) PEX11-BETA
    DNA
    TYPE functioning gene
    STRUCTURE 7.58 kb     4 Exon(s)
    Genomic sequence alignment details
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    4 - 1836 - 259 - 2003 12559946
    4 - 1642 - 245 - 2003 12559946
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Digestivesalivary gland   highly
    Endocrineneuroendocrinepituitary  highly
    Urinarybladder   highly
    Visualeye   highly
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • amphipathic helix (Helix 2) within the first N-terminal 40 AAs is crucial for membrane elongation and self-interaction
  • two transmembrane domains flanking an internal region exposed to the peroxisomal matrix and N- and C-termini facing the cytosol
  • a glycine-rich internal region dispensable for peroxisome membrane elongation and division
    • HOMOLOGY
    Homologene
    FAMILY
  • peroxin-11 family
    • CATEGORY signaling growth factor
    SUBCELLULAR LOCALIZATION extracellular
        intracellular
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,peroxisome
    text integral to peroxisomal membrane
    basic FUNCTION
  • mediating peroxisome proliferation in absence of external stimuli
  • inducing peroxisome division by recruiting dynamin related gtpase to the peroxisome membrane
  • phosphorylation-dependent activation of peroxisome proliferator protein PEX11B controls peroxisome abundance (Knoblach 2010)
    • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • PEX19
  • FIS1 plays important roles in peroxisome division and maintenance of peroxisome morphology in mammalian cells, possibly in a concerted manner with PEX11B and DNM1L
    • cell & other
    REGULATION
    Other self-interaction of PEX11B regulates its membrane deforming activity in conjunction with membrane lipids
    ASSOCIATED DISORDERS
    corresponding disease(s) PBD13
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    corresponding disease(s) PBD13
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
    PEX11 beta deficiency is lethal and impairs neural migration but does not abrogate peroxisome function (Li 2002)