Skip to main content
SpringerLink
Log in

Role of Wnt1 and Fzd1 in the spinal cord pathogenesis of amyotrophic lateral sclerosis-transgenic mice

  • Original Research Paper
  • Published:
Biotechnology Letters Aims and scope Submit manuscript

Abstract

Amyotrophic lateral sclerosis (ALS) is a fatal neurodegenerative disease characterized by chronic progressive degeneration of motor neurons resulting in muscular atrophy, paralysis, and ultimately death. We have investigated the expression of Wnt1 and Fzd1 in the spinal cords of SOD1G93A ALS transgenic mice, SOD1G93A-transfected N2a cells, and primary cultured astrocytes from SOD1G93A transgenic mice. In addition, we provided further insight into the role of Wnt1 and Fzd1 in the pathogenesis of ALS transgenic mice and discuss the mechanisms underlying the Wnt signal pathway which may be useful in the treatment of ALS. The results indicate the involvement of Wnt1 and Fzd1 in the pathogenesis and development of ALS.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7

Similar content being viewed by others

References

  • Carmon KS, Loose DS (2010) Development of a bioassay for detection of Wnt-binding affinities for individual frizzled receptors. Anal Biochem 401:288–294

    Article  PubMed  CAS  Google Scholar 

  • Chen Y, Guan Y, Liu H, Wu X, Yu L, Wang S, Zhao C, Du H, Wang X (2012a) Activation of the Wnt/β-catenin signaling pathway is associated with glial proliferation in the adult spinal cord of ALS transgenic mice. Biochem Biophys Res Commun 420:397–403

    Article  PubMed  CAS  Google Scholar 

  • Chen Y, Guan Y, Zhang Z, Liu H, Wang S, Yu L, Wu X, Wang X (2012b) Wnt signaling pathway is involved in the pathogenesis of amyotrophic lateral sclerosis in adult transgenic mice. Neurol Res 34:390–499

    Article  PubMed  CAS  Google Scholar 

  • Crippa V, Sau D, Rusmini P, Boncoraglio A, Onesto E, Bolzoni E, Galbiati M, Fontana E, Marino M, Carra S, Bendotti C, De Biasi S, Poletti A (2010) The small heat shock protein B8 (HspB8) promotes autophagic removal of misfolded proteins involved in amyotrophic lateral sclerosis (ALS). Hum Mol Genet 19:3440–3456

    Article  PubMed  CAS  Google Scholar 

  • da Cruz e Silva OA, Henriques AG, Domingues SC, da Cruz e Silva EF (2010) Wnt signaling is a relevant pathway contributing to amyloid beta-peptide-mediated neuropathology in Alzheimer’s disease. CNS Neurol Disord: Drug Targets 9:720–726

    Article  Google Scholar 

  • Godin JD, Poizat G, Hickey MA, Maschat F, Humbert S (2010) Mutant huntingtin-impaired degradation of beta-catenin causes neurotoxicity in Huntington’s disease. EMBO J 29:2433–2445

    Article  PubMed  CAS  Google Scholar 

  • Guan YJ, Wang X, Wang HY, Kawagishi K, Ryu H, Huo CF, Shimony EM, Kristal BS, Kuhn HG, Friedlander RM (2007) Increased stem cell proliferation in the spinal cord of adult amyotrophic lateral sclerosis transgenic mice. J Neurochem 102:1125–1138

    Article  PubMed  CAS  Google Scholar 

  • Karbowski M, Neutzner A (2012) Neurodegeneration as a consequence of failed mitochondrial maintenance. Acta Neuropathol 123:157–171

    Article  PubMed  CAS  Google Scholar 

  • Kiernan MC, Vucic S, Cheah BC, Turner MR, Eisen A, Hardiman O, Burrell JR, Zoing MC (2011) Amyotrophic lateral sclerosis. Lancet 377:942–955

    Article  PubMed  CAS  Google Scholar 

  • L’Episcopo F, Serapide MF, Tirolo C, Testa N, Caniglia S, Morale MC, Pluchino S, Marchetti B (2011) A Wnt1 regulated Frizzled-1/β-Catenin signaling pathway as a candidate regulatory circuit controlling mesencephalic dopaminergic neuron-astrocyte crosstalk: therapeutical relevance for neuron survival and neuroprotection. Mol Neurodegener 6:1–29

    Article  Google Scholar 

  • Martin LJ (2012) Biology of mitochondria in neurodegenerative diseases. Prog Mol Biol Transl Sci 107:355–415

    Article  PubMed  CAS  Google Scholar 

  • Marx J (1994) Mouse model found for ALS. Science 264:1663–1664

    Article  PubMed  CAS  Google Scholar 

  • Rosen DR, Siddique T, Patterson D, Figlewicz DA, Sapp P, Hentati A, Donaldson D, Goto J, O’Regan JP, Deng HX (1993) Mutations in Cu/Zn superoxide dismutase gene are associated with familial amyotrophic lateral sclerosis. Nature 362:20–21

    Article  Google Scholar 

  • Wang Y, Thekdi N, Smallwood PM, Macke JP, Nathans J (2002) Frizzled-3 is required for the development of major fiber tracts in the rostral CNS. J Neurosci 22:8563–8573

    PubMed  CAS  Google Scholar 

  • Zhao C, Avilés C, Abel RA, Almli CR, McQuillen P, Pleasure SJ (2005) Hippocampal and visuospatial learning defects in mice with a deletion of frizzled 9, a gene in the Williams syndrome deletion interval. Development 132:2917–2927

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

This work was supported by funds from the National Natural Science Foundation of China (81271413), the Shandong Province Science and Technology Development Program of China (2012GSF11827), the Shandong Province Natural Science Foundation of China (ZR2012HQ021), the Shandong Province Education Department of China (J11LF16), and the Muscular Dystrophy Association, USA (254530 to X. W.). We thank Professor He Li (Tongji Medical College of Huazhong University of Science and Technology) for access to lab facilities and Professor Angelo Poletti (InterUniversity Center on Neurodegenerative Diseases of the Universities of Milano, Genova, Italy) for plasmids.

Author information

Authors and Affiliations

  1. Department of Histology and Embryology, Weifang Medical University, Weifang, Shandong, People’s Republic China

    Shanshan Wang, Yingjun Guan, Yanchun Chen, Xiaojin Li, Caixia Zhang, Li Yu & Fenghua Zhou

  2. Department of Neurosurgery, Brigham and Women’s Hospital, Harvard Medical School, Boston, MA, USA

    Xin Wang

Authors
  1. Shanshan Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Yingjun Guan
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Yanchun Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Xiaojin Li
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Caixia Zhang
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Li Yu
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Fenghua Zhou
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Xin Wang
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Yingjun Guan.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 (DOCX 15 kb)

Rights and permissions

Reprints and permissions

About this article

Cite this article

Wang, S., Guan, Y., Chen, Y. et al. Role of Wnt1 and Fzd1 in the spinal cord pathogenesis of amyotrophic lateral sclerosis-transgenic mice. Biotechnol Lett 35, 1199–1207 (2013). https://doi.org/10.1007/s10529-013-1199-1

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s10529-013-1199-1

Keywords

Search

Navigation