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FLASH GENE
Symbol DHH contributors: mct - updated : 15-10-2018
HGNC name desert hedgehog homolog (Drosophila)
HGNC id 2865
PROTEIN
PHYSICAL PROPERTIES
STRUCTURE
motifs/domains
six cadherin domains
HOMOLOGY
interspecies homolog to murine Dhh
homolog to desert hedgehog Drosophila dhh
Homologene
FAMILY hedgehog family
CATEGORY regulatory , signaling
SUBCELLULAR LOCALIZATION extracellular
    plasma membrane
basic FUNCTION
  • regulating the cardiac development and in the developing of Schwann cells of the peripheral nervous system
  • playing a key role in the differentiation of male gonads
  • combinatorial expression of the paracrine factor DHH and nuclear transcription factor SF1 is required for Leydig cell development
  • is essential for testis development and Schwann cell function
  • DHH promotes peripheral nerve survival in the ischemic muscle and, by doing so, maintains the pool of nerve-derived proangiogenic factors
  • controls the formation of the nerve perineurium during development and is a key component of Schwann cells that ensures peripheral nerve survival
  • pivotal role for DHH in maintaining myelination
  • critical role of DHH in maintaining blood nerve barrier integrity, demonstrating time that endothelial dysfunction is sufficient to induce neuropathy
  • CELLULAR PROCESS
    PHYSIOLOGICAL PROCESS cardiovascular
    PATHWAY
    metabolism
    signaling
  • DHH signaling plays an essential role in the normal development of the testis and in the process of spermatogenesis
  • a component
    INTERACTION
    DNA
    RNA
    small molecule
    protein
  • GLI family in mediating the carcinogenic effect of hedgehog pathway activation
  • SMO (essential component of HH signaling)
  • Sertoli cells coordinate DHH-dependent spermatogenesis events via PTCH1 and SMO prior to the first meiotic division and in postmeiotic (haploid) cells, particularly during the first half of spermiogenesis
  • SOX10 activates DHH expression in Schwann cells via an enhancer that is located in intron 1 of the DHH gene
  • cell & other
    REGULATION
    activated by SOX10
    ASSOCIATED DISORDERS
    corresponding disease(s) GDMN , SRXY7
    Other morbid association(s)
    TypeGene ModificationChromosome rearrangementProtein expressionProtein Function
    constitutional germinal mutation      
    dysgenetic testis, female external genitalia, presence of mullerian structures
    tumoral fusion      
    DHH-RHEBL1 fusion transcript is a novel recurrent feature in the changing landscape of CBFA2T3-GLIS2-positive childhood AML
    Susceptibility
    Variant & Polymorphism
    Candidate gene
    Marker
    Therapy target
    ANIMAL & CELL MODELS
  • mice lacking both Dhh and Ihh had loss of normal ovarian function
  • in Dhh-deficient mice, Dhh negatively regulates multiple stages of erythrocyte differentiation