Cystatin B and SOD1: Protein–Protein Interaction and Possible Relation to Neurodegeneration

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• 作者：Lisa Ulbrich (1)
  Mauro Cozzolino (3) (4)
  Elettra Sara Marini (1)
  Ilaria Amori (3)
  Antonella De Jaco (1)
  Maria Teresa Carrì (2) (3)
  Gabriella Augusti-Tocco (1)
  
• 关键词：Cystatin B ; SOD1 ; fALS ; EPM1
• 刊名：Cellular and Molecular Neurobiology
• 出版年：2014
• 出版时间：March 2014
• 年：2014
• 卷：34
• 期：2
• 页码：205-213
• 全文大小：1,445 KB
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• 作者单位：Lisa Ulbrich (1)
  Mauro Cozzolino (3) (4)
  Elettra Sara Marini (1)
  Ilaria Amori (3)
  Antonella De Jaco (1)
  Maria Teresa Carrì (2) (3)
  Gabriella Augusti-Tocco (1)
  
  1. Department of Biology and Biotechnology “Charles Darwin- University of Rome “Sapienza- P.le A. Moro 5, 00185, Rome, Italy
  3. Santa Lucia Foundation IRCCS, Rome, Italy
  4. Institute of Translational Pharmacology, CNR, Rome, Italy
  2. Department of Biology, University of Rome “Tor Vergata- Rome, Italy
  
• ISSN：1573-6830

文摘

Cystatin B (CSTB), an inhibitor of the cysteine proteases, belongs to the cathepsin family and it is known to interact with a number of proteins involved in cytoskeletal organization. CSTB has an intrinsic tendency to form aggregates depending on the redox environment. The gene encoding for CSTB is frequently mutated in association with the rare neurodegenerative condition progressive myoclonus epilepsy. Increased levels of CSTB have been observed in the spinal cord of transgenic mice modeling SOD1-linked familial amyotrophic lateral sclerosis, a fatal neurodegenerative disease affecting motoneurons. In the present study, we have investigated the relationship occurring between the expression of SOD1 and CSTB either wild-type or double-cysteine substitution mutant (Cys 3 and Cys 64). Whether or not there is a physical interaction between the two proteins was also investigated in overexpression experiments using a human neuroblastoma cell line and mouse-immortalized motoneurons. Here we report evidences for a reciprocal influence of CSTB and SOD1 at the gene expression level and for a direct interaction of the two proteins.