Detection of β-A4 amyloid and its precursor protein in the muscle of a patient with juvenile neuronal ceroid lipofuscinosis (Spielmeyer-Vogt-Sjogren)

M. Villanova, C. Ceuterick, M. T. Dotti, F. M. Santorelli, C. Casali, A. Malandrini, N. De Stefano, U. Lübke, J. J. Martin, G. C. Guazzi, A. Federico

Research output: Contribution to journalArticle

Abstract

Muscle biopsy tissue from a patient affected by the juvenile form of neuronal ceroid lipofuscinosis (NCL) was studied immunohistochemically using antibodies to β-amyloid peptide and amyloid precursor protein. Positive reaction in muscle was specifically localized to autophagic vacuoles and blood vessel walls. Increased acid phosphatase reaction suggested enhanced lysosomal activity. We hypothesize that β-amyloid is deposited in NCL muscle by a lysosomal mechanism similar to that proposed in other disorders involving β-amyloid.

Original languageEnglish
Pages (from-to)78-84
Number of pages7
JournalActa Neuropathologica
Volume98
Issue number1
DOIs
Publication statusPublished - Jul 1999

Keywords

  • β-A4 amyloid
  • Amyloid precursor protein
  • Juvenile neuronal ceroid lipofuscinosis

ASJC Scopus subject areas

  • Clinical Neurology
  • Pathology and Forensic Medicine
  • Neuroscience(all)

Fingerprint Dive into the research topics of 'Detection of β-A4 amyloid and its precursor protein in the muscle of a patient with juvenile neuronal ceroid lipofuscinosis (Spielmeyer-Vogt-Sjogren)'. Together they form a unique fingerprint.

  • Cite this

    Villanova, M., Ceuterick, C., Dotti, M. T., Santorelli, F. M., Casali, C., Malandrini, A., De Stefano, N., Lübke, U., Martin, J. J., Guazzi, G. C., & Federico, A. (1999). Detection of β-A4 amyloid and its precursor protein in the muscle of a patient with juvenile neuronal ceroid lipofuscinosis (Spielmeyer-Vogt-Sjogren). Acta Neuropathologica, 98(1), 78-84. https://doi.org/10.1007/s004010051053