Functional Impairment of the Nervous System with Glycolipid Deficiencies

Yutaka Itokazu, Takahiro Fuchigami, Robert K. Yu

Research output: Chapter in Book/Report/Conference proceedingChapter

1 Scopus citations


Patients with nervous system disorders suffer from impaired cognitive, sensory and motor functions that greatly inconvenience their daily life and usually burdens their family and society. It is difficult to achieve functional recovery for the damaged central nervous system (CNS) because of its limited ability to regenerate. Glycosphingolipids (GSLs) are abundant in the CNS and are known to play essential roles in cell-cell recognition, adhesion, signal transduction, and cellular migration, that are crucial in all phases of neurogenesis. Despite intense investigation of CNS regeneration, the roles of GSLs in neural regeneration remain unclear. Here we focus on the respective potentials of glycolipids to promote regeneration and repair of the CNS. Mice lacking glucosylceramide, lactosylceramide or gangliosides show lethal phenotypes. More importantly, patients with ganglioside deficiencies exhibit severe clinical phenotypes. Further, neurodegenerative diseases and mental health disorders are associated with altered GSL expression. Accumulating studies demonstrate that GSLs not only delimit physical regions but also play central roles in the maintenance of the biological functions of neurons and glia. We anticipate that the ability of GSLs to modulate behavior of a variety of molecules will enable them to ameliorate biochemical and neurobiological defects in patients. The use of GSLs to treat such defects in the human CNS will be a paradigm-shift in approach since GSL-replacement therapy has not yet been achieved in this manner clinically.

Original languageEnglish (US)
Title of host publicationAdvances in Neurobiology
Number of pages30
StatePublished - 2023
Externally publishedYes

Publication series

NameAdvances in Neurobiology
ISSN (Print)2190-5215
ISSN (Electronic)2190-5223


  • Carbohydrate
  • Ganglioside
  • Glycoconjugate
  • Glycolipid
  • Glycosphingolipid
  • Mental health disorder
  • Neural development
  • Neural stem cell
  • Neurodegenerative disease
  • Neurogenesis
  • Neurological disorder
  • Regeneration

ASJC Scopus subject areas

  • Biochemistry
  • Neurology
  • Developmental Neuroscience
  • Cellular and Molecular Neuroscience


Dive into the research topics of 'Functional Impairment of the Nervous System with Glycolipid Deficiencies'. Together they form a unique fingerprint.

Cite this