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FLASH GENE
Symbol ZDHHC17 contributors: mct/npt - updated : 23-02-2015
HGNC name zinc finger, DHHC-type containing 17
HGNC id 18412
Location 12q21.2      Physical location : 77.157.853 - 77.247.474
Synonym name
  • Huntington interactive protein 14
  • Huntingtin interacting protein H
  • palmitoyltransferase ZDHHC17
  • putative NF-kappa-B-activating protein 205
  • huntingtin interacting protein 3
  • related to S.pombe hypothetical 72.5kDa protein C2F7-10
  • Synonym symbol(s) HIP14, HIP3, HYPH, HSPC294, KIAA0946, DHHC17
    EC.number 2.3.1.-
    DNA
    TYPE functioning gene
    STRUCTURE 89.62 kb     17 Exon(s)
    MAPPING cloned Y linked N status provisional
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    17 - 4771 72.51 632 - 2008 18794299
    EXPRESSION
    Type widely
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Cardiovascularheart    
    Endocrineadrenal gland   highly
    Hearing/Equilibriumear   highly
    Nervousbrainhindbraincerebellum highly
     brainbasal nucleistriatum highly
    Respiratoryrespiratory tractlarynx  highly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Epithelialsecretoryglandularendocrine 
    Muscularstriatumskeletal highly
    cell lineage
    cell lines
    fluid/secretion
    at STAGE
    physiological period fetal
    Text liver
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • HIP14 PEP1 domain
  • five ankyrin repeat domains
  • four transmembrane domains
  • DHHC-type zinc finger
  • HOMOLOGY
    interspecies homolog to yeast S. Cerevisiae Akr1p
    intraspecies paralog to ZDHHC13
    Homologene
    FAMILY
  • DHHC palmitoyltransferase family
  • AKR/ZDHHC17 subfamily
  • CATEGORY regulatory , protooncogene
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,membrane
    intracellular,cytoplasm,organelle,Golgi
    intracellular,cytoplasm,cytosolic,vesicle
    text
  • localization within the presynaptic nerve terminal (Stowers 2007)
  • with ZDHHC17, co-localized to the Golgi and the same post-Golgi vesicles, suggesting that heteropalmitoylation might play a physiological role in addition to a biochemical function (Hines 2010)
  • basic FUNCTION
  • playing a role in intracellular trafficking and endocytosis
  • acting as a protein palmitoyltransferase and having an oncogenic potential
  • palmitoyl transferase involved in the palmitoylation and trafficking of multiple neuronal proteins
  • having palmitoyl acyltransferase activity through innate cysteine-rich domains, DHHC (Goytain 2008)
  • encode Mg2+ transport proteins that are regulated by their innate palmitoyl acyltransferases thus fulfilling the characteristics of "chanzymes", proteins combining transport and enzymatic functions (Goytain 2008)
  • controls neurotransmitter release by regulating the trafficking of cysteine string protein to synapses (Ohyama 2007)
  • is the only ZDHHC gene transcribed in normal human erythroid cells
  • is critically involved in behavioral modulation of striatal processing
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS
    PATHWAY
    metabolism
    signaling
    a component
    INTERACTION
    DNA
    RNA
    small molecule metal binding,
  • Zn2+
  • protein
  • binds to HTT and robustly palmitoylates HTT at cysteine 214
  • ZDHHC17 is the major CLIP3 palmitoyltransferase, that modulates CLI3 plasma membrane binding, thereby regulating AKT1 signaling and SLC2A4 membrane translocation in adipocytes
  • it is possible that optineurin regulates trafficking of ZDHHC17 to its substrates
  • GPM6A, and the Sprouty domain-containing proteins SPRED1 and SPRED3, are indeed palmitoylated by ZDHHC17
  • HTT is palmitoylated by ZDHHC17 and 13
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s)
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional       loss of function
    in the absence of ZDHHC17, striatal neurons become dysfunctional, leading to impaired motor behavior
    constitutional     --low  
    lack or marked decrease of the DHHC17 protein might lead to changes in membrane lateral organization
    constitutional     --low  
    loss of palmitoylation of MPP1 attributable to absence of DHHC17 membrane associated with hemolytic anemia
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • mice deficient for Zdhhc17 expression develop neuropathological and behavioural features similar to HD, and the catalytic activity of Zdhhc13 is impaired in HD mice, most likely due to the reduced interaction of Zdhhc17 with Htt
  • loss of Zdhhc17 and Zdhhc13 leads to early embryonic lethality at day embryonic day 10-11 due to failed chorioallantoic fusion