Mar 24, 2009

TDP-43 in skeletal muscle of patients affected with amyotrophic lateral sclerosis

Amyotrophic Lateral Sclerosis : Official Publication of the World Federation of Neurology Research Group on Motor Neuron Diseases
Gianni SorarúCorrado Angelini

Abstract

TAR DNA binding protein (TDP-43) is the pathologic substrate of neuronal and glial aggregates in amyotrophic lateral sclerosis (ALS). Pathologic TDP-43 is hyperphosphorylated and cleaved to generate abnormal protein species that accumulate in the cytoplasm. To assess the hypothesis of TDP-43 pathology as a systemic disorder in ALS we analysed the immunohistochemical and biochemical profile of TDP-43 in muscle biopsies of 30 ALS patients and 30 controls. In all ALS muscle biopsies we observed that TDP-43 was constantly present in an intranuclear localization and TDP-43 Western blotting showed only a 43-KDa band as controls. Our results suggest that TDP-43 pathology is probably confined to the central nervous system in ALS.

  • References9
  • Citations8

References

  • References9
  • Citations8

Mentioned in this Paper

TARDBP gene
Establishment and Maintenance of Localization
Abnormal protein
Western Blotting
TARDBP
Brain
Systemic Disease
Entire Central Nervous System
Immunocytochemistry
Soleus Muscle Structure

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