Lipid thioesters derived from acylated proteins accumulate in infantile neuronal ceroid lipofuscinosis: correction of the defect in lymphoblasts by recombinant palmitoyl-protein thioesterase.

Samankaltaisia teoksia

• Didemnin binds to the protein palmitoyl thioesterase responsible for infantile neuronal ceroid lipofuscinosis.
  Tekijä: Crews, C M, et al.
  Julkaistu: (1996)
• The crystal structure of palmitoyl protein thioesterase 1 and the molecular basis of infantile neuronal ceroid lipofuscinosis
  Tekijä: Bellizzi, John J., et al.
  Julkaistu: (2000)
• Human recombinant palmitoyl protein thioesterase-1 (PPT1) for preclinical evaluation of enzyme replacement therapy for infantile neuronal ceroid lipofuscinosis
  Tekijä: Lu, Jui-Yun, et al.
  Julkaistu: (2009)
• Human palmitoyl protein thioesterase: evidence for lysosomal targeting of the enzyme and disturbed cellular routing in infantile neuronal ceroid lipofuscinosis.
  Tekijä: Hellsten, E, et al.
  Julkaistu: (1996)
• Intravenous high-dose enzyme replacement therapy with recombinant palmitoyl-protein thioesterase reduces visceral lysosomal storage and modestly prolongs survival in a preclinical mouse model of infantile neuronal ceroid lipofuscinosis
  Tekijä: Hu, Jie, et al.
  Julkaistu: (2012)