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Cystatin C is differentially involved in multiple system atrophy phenotypes.

Aintzane Urbizu1, Julia Canet-Pons1, Ana M Munoz-Marmol1

  • 1Servicio de Anatomía Patológica, Instituto de Investigación en Ciencias de la Salud Germans Trias i Pujol, Hospital Universitario Germans Trias i Pujol, Barcelona, Spain.

Neuropathology and Applied Neurobiology
|March 11, 2014
PubMed
Summary

Cystatin C (CysC) levels are altered in multiple system atrophy (MSA), particularly in parkinsonian (MSAp) and cerebellar (MSAc) subtypes. These CysC changes suggest a role in MSA pathogenesis.

Keywords:
MSA phenotypecathepsin B expressioncathepsin D expressioncathepsin L1 expressioncystatin C expressionmultiple system atrophy (MSA)

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Area of Science:

  • Neuroscience
  • Genetics
  • Biochemistry

Background:

  • Cystatin C (CysC) is implicated in neurodegenerative diseases.
  • Multiple system atrophy (MSA) is a progressive neurodegenerative disorder with parkinsonian (MSAp) and cerebellar (MSAc) phenotypes.

Purpose of the Study:

  • To investigate the relationship between CysC and MSA, including its subtypes.
  • To explore the role of CysC in the pathogenesis of MSA.

Main Methods:

  • Determined Cystatin C gene (CST3) haplotypes in MSA patients and controls.
  • Quantified CST3 and cathepsin mRNA levels in post-mortem brain samples.
  • Performed CysC immunohistochemistry on cerebellar tissues.

Main Results:

  • The CST3 B-haplotype was associated with MSAp.
  • Elevated CST3 mRNA levels were observed in MSAp caudate nuclei and MSAc cerebella.
  • CysC was over-expressed in specific cerebellar cell types in MSAc.

Conclusions:

  • CysC alterations are differentially found in MSA subtypes.
  • CysC may play a significant role in the development of MSA.