Metabolic Brain Disease

, 26:299

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 Luo
Original Paper

DOI: 10.1007/s11011-011-9263-9

Cite this article as:
Yan, X., Lukas, J., Witt, M. et al. Metab Brain Dis (2011) 26: 299. doi:10.1007/s11011-011-9263-9

Abstract

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.

Keywords

NPC1OligodendrocyteMyelinationMyelin gene regulatory factor

Abbreviations

CB

Cerebellum

CNPase

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

CNS

Central nervous system

DH

Dorsal horn

E

Embryonic day

EC

Olfactory ensheathing cells

EPI

External plexiform layer

E/OV

Extended amygdale and olfactory ventricle

ER

Endoplasmic reticulum

FB

Forebrain

GAPDH

Glyceraldehyde 3-phosphate dehydrogenase

Gl

Glomerular layer

GrO

Granule cell layer

HB

Hindbrain

IPI

Internal plexiform layer

MBP

Myelin basic protein

MI

Mitral cell layer

MRF

Myelin gene regulatory factor

NPC

Niemann-Pick type C

NPC1−/−

NPC1-mutant

OB

Olfactory bulb

OLs

Myelinating oligdendrocytes

OPCs

Oligodendrocyte progenitor cells

OPLs

Postmitotic premyelinating oligodendrocytes

P

Postnatal day

SOX10

SRY-related HMG-box 10

SP

Spinal cord

VH

Ventral horn

YY1

Transcription factor Yin Yang 1

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
  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