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Ontology Term : Dejerine-Sottas syndrome UniProtKeyword

description  Protein which, if defective, causes Dejerine-Sottas disease. DSS is a hereditary motor and sensory neuropathy (HMSN) of the Charcot-Marie- Tooth disease type 1 class. DSS is characterized by severe early onset, very slow nerve conduction velocities (less than 12m/sec) and raised cerebrospinal fluid protein concentrations (0.7 g/l). Clinical signs are delayed age of walking as well as areflexia
  • synonyms:
  • DSS
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