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FLASH GENE
Symbol GOLPH3 contributors: shn/npt - updated : 05-06-2014
HGNC name golgi phosphoprotein 3 (coat-protein)
HGNC id 15452
Location 5p13.3      Physical location : 32.124.823 - 32.174.425
Synonym name
  • golgi peripheral membrane protein 1, 34 kDa
  • golgi protein
  • coat-protein
  • golgi-associated protein
  • golgi associated protein, 34-KD
  • mitochondrial DNA absence-sensitive factor
  • Synonym symbol(s) GPP34, GOPP1, MIDAS, FLJ90675, GMx33
    DNA
    TYPE functioning gene
    STRUCTURE 49.60 kb     4 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    MAPPING cloned Y linked N status confirmed
    Map pter - D5S477 - D5S1996 - GOLPH3 - D5S1470 - D5S2861 - cen
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    4 - 2676 34 298 - 2005 16263763
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • a cluster of arginine residues at the N-terminal end of GOLPH3 proteins that are necessary and sufficient to mediate coatomer binding
  • a PtdIns(4)P binding domain
  • HOMOLOGY
    interspecies ortholog to Golph3, Mus musculus
    ortholog to Golph3, Rattus norvegicus
    ortholog to GOLPH3, Pan troglodytes
    ortholog to golph3, Danio rerio
    intraspecies paralog to GOLPH3L
    Homologene
    FAMILY
  • GOLPH3/VPS74 family
  • CATEGORY structural protein , protooncogene , transport
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria,interspace
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,Golgi
    intracellular,cytoplasm,organelle,endosome
    intracellular,cytoplasm,cytosolic
    text periphery of Golgi stacks, on the cis and trans sides and at the lateral edges of stacked cisternae (Bell et al, 2001)
    basic FUNCTION
  • playing a role for a novel coat protein in Golgi trafficking
  • involved in the regulation of mitochondrial lipids, leading to increases of total mitochondrial mass in response to mitochondrial dysfunction (Nakashima-Kamimura et al, 2005)
  • regulates cell size, enhances growth-factor-induced mTOR signalling in human cancer cells, and alters the response to an mTOR inhibitor in vivo (Scott et al, 2009)
  • necessary for extension of the Golgi around the nucleus (Dippold et al, 2009)
  • phosphatidylinositol-4-phosphate (PI4P) effector that plays an important role in maintaining Golgi architecture and anterograde trafficking
  • contributes to the motility of glioma cells by regulating the expression of RHOA
  • importance of GOLPH3 in vesicular trafficking, Golgi architecture maintenance, receptor sorting, protein glycosylation
  • in premeiotic spermatocytes GOLPH3 protein is required for maintaining the organization of Golgi stacks, and in dividing spermatocytes is essential for both contractile ring and central spindle formation during cytokinesis
  • acts as a key molecule to coordinate phosphoinositide signaling with actomyosin dynamics and vesicle trafficking during cytokinesis
  • role of GOLPH3 in N-glycosylation to regulate cell biological functions
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS cellular trafficking transport
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule other,
  • phosphatidylinositol-4-phosphate (PtdIns(4)P)
  • PtdIns(4)P-rich trans-Golgi membranes
  • protein
  • myosin XVIIIA, MYO18A (Dippold et al, 2009)
  • GOLPH3L antagonizes GOLPH3/MYO18A at the Golgi
  • DNA-damage-induced Golgi dispersal requires GOLPH3/MYO18A/F-actin and the DNA damage protein kinase, PRKDC
  • GOLPH3 was found to bind to POMGNT1 which is involved in O-mannosylation of alpha-dystroglycan (novel role for GOLPH3 in mediating the Golgi localization of POMGNT1)
  • specific interaction between sialyltransferases and GOLPH3 important for the sialylation
  • PRKD2 promotes glioma cell proliferation by regulating GOLPH3 and then AKT activation
  • cell & other
  • binds directly and specifically to Golgi membranes through PtdIns(4)P Dippold et al, 2009)
  • REGULATION
    Other enhanced by the absence of mitochondrial DNA (Nakashima-Kamimura et al, 2005)
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral     --over  
    in esophageal squamous cell carcinoma correlates with poor prognosis
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
  • prognostic biomarker for RCC (renal cell carcinoma)
  • Therapy target
    SystemTypeDisorderPubmed
    cancerurinary 
    promising therapeutic target for RCC (renal cell carcinoma)
    cancerbrainglioma/neuroblstoma
    PRKD2-GOLPH3-AKT signaling pathway might be a potential glioma therapeutic target
    ANIMAL & CELL MODELS
  • Knockdown of GOLPH3 in HeLa cells results in compaction of the Golgi without altering F-actin (Scott et al, 2009)