Metabolic Brain Disease

, 26:299 | Cite as

Decreased expression of myelin gene regulatory factor in Niemann-Pick type C 1 mouse

  • Xin Yan
  • Jan Lukas
  • Martin Witt
  • Andreas Wree
  • Rayk Hübner
  • Moritz Frech
  • Rüdiger Köhling
  • Arndt Rolfs
  • Jiankai LuoEmail author
Original Paper


Niemann-Pick type C 1 (NPC1) disease is an autosomal recessive cholesterol transport defect resulting in a neurodegenerative process in patients mainly at an early age, although some patients may start with manifestation in adult. Since loss of myelin is considered as a main pathogenetic factor, the precise mechanism inducing dysmylination in NPC1 disease is still unclear. In the present study, a quantitative evaluation on the myelin protein and its regulatory factors of oligodendrocytes, such as SRY-related HMG-box 10 (Sox10), Yin Yang 1 factor (YY1) and myelin gene regulatory factor (MRF), in different parts of the brain and spinal cord was performed in NPC1-mutant mice. The results showed that NPC1 protein was expressed in oligodendrocytes and the amount of myelin protein was generally decreased in all parts of the brain and spinal cord in NPC1-mutant mice. Compared to wild type, the amount of Sox10 and YY1 was not different in NPC1-mutant mice, but MRF was significantly decreased, suggesting a possible mechanism perturbing differentiation of oligodendrocytes and the myelination process in the NPC1-mutant mouse.


NPC1 Oligodendrocyte Myelination Myelin gene regulatory factor 





2′,3′-cyclic nucleotide-3′-phosphodiesterase


Central nervous system


Dorsal horn


Embryonic day


Olfactory ensheathing cells


External plexiform layer


Extended amygdale and olfactory ventricle


Endoplasmic reticulum




Glyceraldehyde 3-phosphate dehydrogenase


Glomerular layer


Granule cell layer




Internal plexiform layer


Myelin basic protein


Mitral cell layer


Myelin gene regulatory factor


Niemann-Pick type C




Olfactory bulb


Myelinating oligdendrocytes


Oligodendrocyte progenitor cells


Postmitotic premyelinating oligodendrocytes


Postnatal day


SRY-related HMG-box 10


Spinal cord


Ventral horn


Transcription factor Yin Yang 1



This work is supported by an intramural FORUN program from School of Medicine University of Rostock.


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Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Xin Yan
    • 1
  • Jan Lukas
    • 1
  • Martin Witt
    • 2
  • Andreas Wree
    • 2
  • Rayk Hübner
    • 1
  • Moritz Frech
    • 1
  • Rüdiger Köhling
    • 3
  • Arndt Rolfs
    • 1
  • Jiankai Luo
    • 1
    Email author
  1. 1.Albrecht-Kossel-Institute for Neuroregeneration, School of MedicineUniversity of RostockRostockGermany
  2. 2.Institute of Anatomy, School of MedicineUniversity of RostockRostockGermany
  3. 3.Oscar Langendorff Institute of Physiology, School of MedicineUniversity of RostockRostockGermany

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