Myelin turnover in hyperphenylalaninaemia. A re-evaluation with the HPH-5 mouse

J Inherit Metab Dis. 1992;15(2):243-51. doi: 10.1007/BF01799638.


Myelin turnover has been studied in the 25-day-old HPH-5 mouse, a phenylalanine hydroxylase-deficient mouse mutant. The half-life of the fast component of myelin decreased from 15 days in control mice to 4.5 days at blood phenylalanine levels of 2.5 mmol/L. The slow component of myelin seems also to be affected by the high phenylalanine level. These observations confirm similar observations obtained with chemically induced models of hyperphenylalaninaemia and are therefore due to the hyperphenylalaninaemia per se, independently of the inhibitors of phenylalanine hydroxylase. An intermediate blood level of phenylalanine (0.7 mmol/L) likewise seems to interfere with myelin metabolism, although to a lesser degree.

MeSH terms

  • Aging
  • Amino Acids / blood
  • Amino Acids / metabolism
  • Animals
  • Body Weight
  • Brain / metabolism
  • Brain / pathology
  • Half-Life
  • Mice
  • Mice, Mutant Strains
  • Myelin Proteins / metabolism
  • Myelin Sheath / metabolism*
  • Organ Size
  • Phenylalanine / administration & dosage
  • Phenylalanine / blood*
  • Phenylalanine Hydroxylase / deficiency*


  • Amino Acids
  • Myelin Proteins
  • Phenylalanine
  • Phenylalanine Hydroxylase