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A CLN5 mutation causing an atypical neuronal ceroid lipofuscinosis of juvenile onset (2005)
  • Pineda-Trujillo,
  • N.,
  • Cornejo,
  • W.,
  • Carrizosa,
  • J.,
  • Wheeler,
  • R.B.,
  • Munera,
  • S.,
  • Valencia,
  • A.,
  • Agudelo-Arango,
  • J.,
  • Cogollo,
  • A.,
  • Anderson,
  • G.,
  • Bedoya,
  • G.,
  • Mole,
  • S.E.,
  • Ruiz-Linares,
  • A.

Abstract
Three related patients from Colombia presented with a juvenile- onset neuronal ceroid lipofuscinosis. Electron microscopy of one case showed condensed fingerprint profiles, and genetic analyses identified a novel missense mutation in CLN5. The authors demonstrate the existence of pathogenic CLN5 mutations outside northern Europe and that mutations in this gene can lead to an atypical late-onset neuronal ceroid lipofuscinosis disease, in addition to the late infantile form first described in Finland

Publication details
Repository UCL Research Publications Index (MyOPIA) (United Kingdom)
Keywords ,A, AND, CLN5, DISEASE, electron microscopy, Europe, FORM, GENE, Genetic, Infantile, JOURNAL, lipofuscinosis, Microscopy, MISSENSE MUTATION, MUTATION, MUTATIONS, Neurology, NEURONAL CEROID LIPOFUSCINOSIS, OF, ONSET, PATIENT, patients, PROFILES, THE
Type JOUR
Relation 740-742