Patterns of abnormal protein expression in target formations and unstructured cores

Jan L. De Bleecker, Birgit B. Ertl, Andrew G Engel

Research output: Contribution to journalArticle

26 Citations (Scopus)

Abstract

Streaming of Z-disks and focal myofibrillar degeneration occur both in target formations (TF) and unstructured cores (UC). Similar myofibrillar alterations are also part of the spectrum of ultrastructural reactions that can occur in the myopathies associated with myofibrillar degeneration and abnormal foci of desmin positivity. In the latter disorders, there is ectopic overexpression of dystrophin, neural cell adhesion molecule (NCAM), gelsolin, β-amyloid precursor protein (βAPP) epitopes, α1-antichymotrypsin (α1-ACT), and many abnormal fiber regions are also strongly congophilic. Therefore, we searched for similar abnormalities in TF and UC. The UC and the center of TF show increased immunoreactivity for actin, α-actinin, gelsolin, dystrophin, βAPP epitopes, (α1-ACT, β2-microglobulin, desmin, and NCAM, but minimal or no congophilia. The periphery of the TF reacts strongly for nebulin but not for actin. The observed immunocytochemical alterations in TF and UC may represent a stereotyped cellular response associated with myofibrillar degeneration due to any cause. However, the three-dimensional profile of the TF and UC as well as their fiber-type specificity distinguish them from lesions that have similar immunocytochemical profiles in other myopathies.

Original languageEnglish (US)
Pages (from-to)339-349
Number of pages11
JournalNeuromuscular Disorders
Volume6
Issue number5
DOIs
StatePublished - Oct 1996

Fingerprint

Gelsolin
Neural Cell Adhesion Molecules
Dystrophin
Desmin
Amyloid beta-Protein Precursor
Muscular Diseases
Actins
Epitopes
Actinin
Proteins
nebulin

Keywords

  • β-amyloid precursor protein
  • Central core
  • Cytoskeletal proteins
  • Desmin
  • Dystrophin
  • Target formation

ASJC Scopus subject areas

  • Clinical Neurology
  • Pediatrics, Perinatology, and Child Health
  • Developmental Neuroscience
  • Neurology

Cite this

Patterns of abnormal protein expression in target formations and unstructured cores. / De Bleecker, Jan L.; Ertl, Birgit B.; Engel, Andrew G.

In: Neuromuscular Disorders, Vol. 6, No. 5, 10.1996, p. 339-349.

Research output: Contribution to journalArticle

De Bleecker, Jan L. ; Ertl, Birgit B. ; Engel, Andrew G. / Patterns of abnormal protein expression in target formations and unstructured cores. In: Neuromuscular Disorders. 1996 ; Vol. 6, No. 5. pp. 339-349.
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