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FLASH GENE
Symbol FANCG contributors: mct - updated : 13-02-2011
HGNC name Fanconi anemia, complementation group G
HGNC id 3588
Corresponding disease
FANCG Fanconi anemia, complementation group G
Location 9p13.3      Physical location : 35.073.834 - 35.080.013
Synonym name
  • Fanconi anemia, complementation group G
  • repair X-ray defect, complementing defect in CHO UV40 cells 9
  • DNA repair protein XRCC9
  • X-ray repair, complementing defective, in Chinese hamster, 9
  • protein FACG
  • X-ray repair complementing defective repair in Chinese hamster cells 9
  • Synonym symbol(s) XRCC9, FAG, FACG
    DNA
    TYPE functioning gene
    STRUCTURE 6.18 kb     14 Exon(s)
    10 Kb 5' upstream gene genomic sequence study
    regulatory sequence cytosine-phosphate-guanine/HTF
    MAPPING cloned Y linked N status provisional
    Map pter - D9S1817 - D9S1805 - FANCG FANCG - D9S1804 - D9S1794 - cen
    Physical map
    C9orf23 9p11.2 chromosome 9 open reading frame 23 DCTN3 9p13 dynactin 3 (p22) LOC138715 9p13.2 similar to E2F binding protein OPRS1 9 opioid receptor, sigma 1 GALT 9p13.3 galactose-1-phosphate uridylyltransferase IL11RA 9p13 interleukin 11 receptor, alpha CCL27 9p13 chemokine (C-C motif) ligand 27 LOC389714 9 LOC389714 CCL19 9p13 chemokine (C-C motif) ligand 19 CCL21 9p13 chemokine (C-C motif) ligand 21 LOC259308 9p12 DKFZP434J193-like pseudogene LOC389715 9 hypothetical gene supported by AK128180 LOC392305 9 similar to GLUL protein LOC158383 9p13.2 tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein, zeta pseudogene KIAA1045 9p13.2 KIAA1045 DNAJB5 9p11.2 DnaJ (Hsp40) homolog, subfamily B, member 5 LOC392306 9 similar to GCIP-interacting protein p29 LOC138724 VCP 9p13-p12 valosin-containing protein FANCG 9p13 Fanconi anemia, complementation group G PIGO 9p13.2 phosphatidylinositol glycan, class O STOML2 9p13 stomatin (EPB72)-like 2 FLJ11560 9p13.2 hypothetical protein FLJ11560 LOC347241 9p13.2 similar to Zinc finger protein 492 UNC13B 9p12-p11 unc-13 homolog B (C. elegans) PRO0038 9p13.2 PRO0038 protein
    RNA
    TRANSCRIPTS type messenger
    identificationnb exonstypebpproduct
    ProteinkDaAAspecific expressionYearPubmed
    14 - 2649 68.6 622 - -
    EXPRESSION
    Type ubiquitous
       expressed in (based on citations)
    organ(s)
    SystemOrgan level 1Organ level 2Organ level 3Organ level 4LevelPubmedSpeciesStageRna symbol
    Lymphoid/Immunethymus   highly
    Reproductivefemale systemovary  highly
     female systemuteruscervix highly
     male systemtestis  highly
    tissue
    SystemTissueTissue level 1Tissue level 2LevelPubmedSpeciesStageRna symbol
    Blood / hematopoieticbone marrow  highly
    Lymphoid    
    cell lineage lymphoblasts
    cell lines
    fluid/secretion
    at STAGE
    PROTEIN
    PHYSICAL PROPERTIES
    STRUCTURE
    motifs/domains
  • N terminal two-thirds of FANCG binds to the N terminal NLS of FANCA
  • two putative leucine zipper domains at the N terminus
  • a motif that interacts directly with the SH3 domain of SPTAN1
  • seven tetratricopeptide repeat motifs
  • conjugated PhosphoP
    mono polymer complex
    HOMOLOGY
    interspecies ortholog to Fancg, Mus musculus
    ortholog to fancg, Danio rerio
    ortholog to Fancg, Rattus norvegicus
    ortholog to FANCG, Pan troglodytes
    Homologene
    FAMILY
    CATEGORY regulatory
    SUBCELLULAR LOCALIZATION     intracellular
    intracellular,cytoplasm,organelle,mitochondria
    intracellular,nucleus,chromatin/chromosome
    text
  • found in mitochondria
  • basic FUNCTION
  • putatively involved in DNA-post replication repair or cell cycle checkpoint control
  • may have a role in protection against oxidative DNA damage
  • protection of the genomic integrity of cells and maintenance of normal chromosome stability
  • required to prevent accumulation of replication-associated DNA double-strand breaks
  • Fanconi anemia proteins, including FANCG are functionally involved in several complex cellular pathways including transcription regulation, cell signaling, oxidative metabolism, and cellular transport
  • predicted to play a key role in the assembly and/or stabilization of the nuclear FA protein core complex
  • phosphorylation of serine 7 in FANCG is functionally important in the FA pathway
  • may have a role independent of the FA core complex
  • a critical role in neural stem and progenitor cells during developmental and adult neurogenesis
  • promotes formation of a newly identified protein complex containing BRCA2, FANCD2 and XRCC3
  • CELLULAR PROCESS cell cycle
    nucleotide, repair
    nucleotide, genomic integrity
    PHYSIOLOGICAL PROCESS development , neurogenesis
    text cell cycle control
    PATHWAY
    metabolism
    signaling
    chromosome instability pathway
    a component
  • component of a nuclear complex with FANCA, FANCC, FANCF
  • member of the Group I Fanconi anemia proteins including also FANCA, FANCB, FANCC, FANCE, FANCF, FANCM, FANCL
  • INTERACTION
    DNA
  • DNA containing psoralen interstrand cross-links
  • RNA
    small molecule
    protein
  • cytochrome P450, family 2, subfamily E, polypeptide 1, CYP2E1
  • breast cancer 2, early onset, BRCA2 and Fanconi anemia, complementation group D1, FANCD1
  • FANCA, FANCC, FANCF and FANCE
  • Protein kinase regulated by RNA, PKR
  • mitochondrial peroxidase peroxiredoxin-3, PRDX3
  • X-ray repair complementing defective repair in Chinese hamster cells 3, XRCC3
  • hairy enhancer of split 1, HES1
  • binding of FANCG to SPTAN1 may be important for the stability of SPTAN1 in cells and the role SPTAN1 plays in the DNA repair process
  • ERCC1-XPF endonuclease
  • cell & other
    REGULATION
    ASSOCIATED DISORDERS
    corresponding disease(s) FANCG
    related resource Fanconi Anaemia Mutation Database
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    tumoral       loss of function
    in leukemia
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • Fancg(-/-) mice have normal viability and no gross developmental abnormalities but their primary splenic lymphocytes, bone marrow progenitor cells, and fibroblasts display spontaneous chromosome breakage and increased sensitivity to mitomycin C and ionizing radiation. Fancg(-/-) mice have decreased fertility and abnormal gonadal histology
  • Fancg/Xrcc9 null mice show showed hypogonadism and impaired fertility and hypersensitivity to mitomycin C
  • fancg(-/-) mice presented with microcephalies and a decreased neuronal production in developing cortex and adult brain