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GJB2

MIM.121011

Pathology

- GJB2 mutations in

  • sensorineural deafness
    • type I autosomal recessive neurosensory deafness (DFNB1) (MIM.220290)
    • autosomal dominant deafness (DFNA3) (MIM.601544)
  • cutaneous disorders-deafness association
    • autosomal dominant keratitis-ichtyosis-deafness syndrome (MIM.148210)
    • palmoplantar keratoderma with deafness (MIM.148350)
    • Bart-Pumphrey syndrome
  • cutaneous disorders
    • hystrix-like ichthyosis (MIM.602540)
    • mutilating keratoderma with ichthyosis (Vohwinkel syndrome with ichtyosis)(MIM.604117)
    • follicular occlusion triad
      • dissecting folliculitis of the scalp
      • hidradenitis suppurativa
      • cystic acne