Advertisement

Schwann Cells pp 359-369 | Cite as

Analysis of Myelinating Schwann Cells in Human Skin Biopsies

  • Mario A. SaportaEmail author
  • Renata de Moraes Maciel
Protocol
Part of the Methods in Molecular Biology book series (MIMB, volume 1739)

Abstract

The human skin is richly innervated by nerve fibers of different calibers and functions, including thickly myelinated large fibers that act as afferents for mechanoreceptors in the dermal papillae. Skin biopsies offer minimally invasive access to these myelinated fibers, in which each internode represents an individual myelinating Schwann cell. Using this approach, human myelinated nerve fibers can be analyzed by several methods, including immunostaining, morphometric and ultrastructural analysis, and molecular biology techniques. This analysis can reveal important aspects of human Schwann cell biology in health and disease, such as in the case of demyelinating neuropathies. This technique has revealed Schwann cell phenotypes in Charcot-Marie-Tooth disease type 1 and acquired inflammatory neuropathies.

Key words

Skin biopsy Immunohistochemistry Myelinated nerve fibers Schwann cells Charcot-Marie-Tooth disease Neuropathy 

Notes

Acknowledgements

The authors would like to thank Dr. Jun Li and Michael Shy for mentoring and training in the performing of skin biopsy and dermal myelinated nerve fiber analysis.

References

  1. 1.
    Polydefkis M, Yiannoutsos CT, Cohen BA et al (2002) Reduced intraepidermal nerve fiber density in HIV-associated sensory neuropathy. Neurology 58:115–119CrossRefPubMedGoogle Scholar
  2. 2.
    Polydefkis M, Hauer P, Griffin JW, McArthur JC (2001) Skin biopsy as a tool to assess distal small fiber innervation in diabetic neuropathy. Diabetes Technol Ther 3:23–28CrossRefPubMedGoogle Scholar
  3. 3.
    Holland NR, TO C, Hauer P et al (1998) Small-fiber sensory neuropathies: clinical course and neuropathology of idiopathic cases. Ann Neurol 44:47–59.  https://doi.org/10.1002/ana.410440111 CrossRefPubMedGoogle Scholar
  4. 4.
    England JD, Gronseth GS, Franklin G et al (2009) Practice parameter: evaluation of distal symmetric polyneuropathy: role of laboratory and genetic testing (an evidence-based review). Report of the American Academy of Neurology, American Association of Neuromuscular and Electrodiagnostic Medicine, and American Academy of Physical Medicine and Rehabilitation. Neurology 72:185–192.  https://doi.org/10.1212/01.wnl.0000336370.51010.a1 CrossRefPubMedGoogle Scholar
  5. 5.
    Nolano M, Provitera V, Crisci C et al (2003) Quantification of myelinated endings and mechanoreceptors in human digital skin. Ann Neurol 54:197–205.  https://doi.org/10.1002/ana.10615 CrossRefPubMedGoogle Scholar
  6. 6.
    Provitera V, Nolano M, Pagano A et al (2007) Myelinated nerve endings in human skin. Muscle Nerve 35:767–775.  https://doi.org/10.1002/mus.20771 CrossRefPubMedGoogle Scholar
  7. 7.
    Li J, Bai Y, Ghandour K et al (2005) Skin biopsies in myelin-related neuropathies: bringing molecular pathology to the bedside. Brain 128:1168–1177.  https://doi.org/10.1093/brain/awh483 CrossRefPubMedGoogle Scholar
  8. 8.
    Sabet A, Li J, Ghandour K et al (2006) Skin biopsies demonstrate MPZ splicing abnormalities in Charcot-Marie-Tooth neuropathy 1B. Neurology 67:1141–1146.  https://doi.org/10.1212/01.wnl.0000238499.37764.b1 CrossRefPubMedGoogle Scholar
  9. 9.
    Saporta MA, Katona I, Lewis RA et al (2009) Shortened internodal length of dermal myelinated nerve fibres in Charcot-Marie-Tooth disease type 1A. Brain 132:3263–3273.  https://doi.org/10.1093/brain/awp274 CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Katona I, Wu X, Feely SME et al (2009) PMP22 expression in dermal nerve myelin from patients with CMT1A. Brain 132:1734–1740.  https://doi.org/10.1093/brain/awp113 CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Doppler K, Werner C, Sommer C (2013) Disruption of nodal architecture in skin biopsies of patients with demyelinating neuropathies. J Peripher Nerv Syst 18:168–176.  https://doi.org/10.1111/jns5.12023 CrossRefPubMedGoogle Scholar
  12. 12.
    Dyck PJ, Gutrecht JA, Bastron JA et al (1968) Histologic and teased-fiber measurements of sural nerve in disorders of lower motor and primary sensory neurons. Mayo Clin Proc 43:81–123PubMedGoogle Scholar
  13. 13.
    Saporta MA, Katona I, Zhang X et al (2011) Neuropathy in a human without the PMP22 gene. Arch Neurol 68:814–821.  https://doi.org/10.1001/archneurol.2011.110 CrossRefPubMedPubMedCentralGoogle Scholar
  14. 14.
    Lee G, Xiang Z, Brannagan TH et al (2010) Differential gene expression in chronic inflammatory demyelinating polyneuropathy (CIDP) skin biopsies. J Neurol Sci 290:115–122.  https://doi.org/10.1016/j.jns.2009.10.006 CrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media, LLC 2018

Authors and Affiliations

  1. 1.Department of NeurologyUniversity of Miami Miller School of MedicineMiamiUSA
  2. 2.Department of Human GeneticsUniversity of Miami Miller School of MedicineMiamiUSA

Personalised recommendations