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

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...

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Published in:Cellular and molecular neurobiology 2014-03, Vol.34 (2), p.205-213
Main Authors: Ulbrich, Lisa, Cozzolino, Mauro, Marini, Elettra Sara, Amori, Ilaria, De Jaco, Antonella, Carrì, Maria Teresa, Augusti-Tocco, Gabriella
Format: Article
Language:English
Subjects:
Animals
Biomedical and Life Sciences
Biomedicine
Blotting, Western
Cell Biology
Cell Line
Clone Cells
Cystatin B - genetics
Cystatin B - metabolism
Gene Expression Regulation
Humans
Immunoprecipitation
Mice
Mutant Proteins - metabolism
Nerve Degeneration - metabolism
Nerve Degeneration - pathology
Neurobiology
Neurosciences
Original Research
Protein Binding
Rats
Solubility
Superoxide Dismutase - genetics
Superoxide Dismutase - metabolism
Superoxide Dismutase-1
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Summary: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.
ISSN:0272-4340
1573-6830
1573-6830
DOI:10.1007/s10571-013-0004-y