Podocyte Injury Associated with Mutant α-Actinin-4
Joint Authors
Cybulsky, Andrey V.
Kennedy, Chris R. J.
Source
Journal of Signal Transduction
Issue
Vol. 2011, Issue 2011 (31 Dec. 2011), pp.1-11, 11 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2011-07-26
Country of Publication
Egypt
No. of Pages
11
Main Subjects
Abstract EN
Focal segmental glomerulosclerosis (FSGS) is an important cause of proteinuria and nephrotic syndrome in humans.
The pathogenesis of FSGS may be associated with glomerular visceral epithelial cell (GEC; podocyte) injury, leading to apoptosis, detachment, and “podocytopenia”, followed by glomerulosclerosis.
Mutations in α-actinin-4 are associated with FSGS in humans.
In cultured GECs, α-actinin-4 mediates adhesion and cytoskeletal dynamics.
FSGS-associated α-actinin-4 mutants show increased binding to actin filaments, compared with the wild-type protein.
Expression of an α-actinin-4 mutant in mouse podocytes in vivo resulted in proteinuric FSGS.
GECs that express mutant α-actinin-4 show defective spreading and motility, and such abnormalities could alter the mechanical properties of the podocyte, contribute to cytoskeletal disruption, and lead to injury.
The potential for mutant α-actinin-4 to injure podocytes is also suggested by the characteristics of this mutant protein to form microaggregates, undergo ubiquitination, impair the ubiquitin-proteasome system, enhance endoplasmic reticulum stress, and exacerbate apoptosis.
American Psychological Association (APA)
Cybulsky, Andrey V.& Kennedy, Chris R. J.. 2011. Podocyte Injury Associated with Mutant α-Actinin-4. Journal of Signal Transduction،Vol. 2011, no. 2011, pp.1-11.
https://search.emarefa.net/detail/BIM-481020
Modern Language Association (MLA)
Cybulsky, Andrey V.& Kennedy, Chris R. J.. Podocyte Injury Associated with Mutant α-Actinin-4. Journal of Signal Transduction No. 2011 (2011), pp.1-11.
https://search.emarefa.net/detail/BIM-481020
American Medical Association (AMA)
Cybulsky, Andrey V.& Kennedy, Chris R. J.. Podocyte Injury Associated with Mutant α-Actinin-4. Journal of Signal Transduction. 2011. Vol. 2011, no. 2011, pp.1-11.
https://search.emarefa.net/detail/BIM-481020
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-481020