Protein Homeostasis Defects of Alanine-Glyoxylate Aminotransferase: New Therapeutic Strategies in Primary Hyperoxaluria Type I
Joint Authors
Pey, Angel L.
Albert, Armando
Salido, Eduardo
Source
Issue
Vol. 2013, Issue 2013 (31 Dec. 2013), pp.1-15, 15 p.
Publisher
Hindawi Publishing Corporation
Publication Date
2013-07-16
Country of Publication
Egypt
No. of Pages
15
Main Subjects
Abstract EN
Alanine-glyoxylate aminotransferase catalyzes the transamination between L-alanine and glyoxylate to produce pyruvate and glycine using pyridoxal 5′-phosphate (PLP) as cofactor.
Human alanine-glyoxylate aminotransferase is a peroxisomal enzyme expressed in the hepatocytes, the main site of glyoxylate detoxification.
Its deficit causes primary hyperoxaluria type I, a rare but severe inborn error of metabolism.
Single amino acid changes are the main type of mutation causing this disease, and considerable effort has been dedicated to the understanding of the molecular consequences of such missense mutations.
In this review, we summarize the role of protein homeostasis in the basic mechanisms of primary hyperoxaluria.
Intrinsic physicochemical properties of polypeptide chains such as thermodynamic stability, folding, unfolding, and misfolding rates as well as the interaction of different folding states with protein homeostasis networks are essential to understand this disease.
The view presented has important implications for the development of new therapeutic strategies based on targeting specific elements of alanine-glyoxylate aminotransferase homeostasis.
American Psychological Association (APA)
Pey, Angel L.& Albert, Armando& Salido, Eduardo. 2013. Protein Homeostasis Defects of Alanine-Glyoxylate Aminotransferase: New Therapeutic Strategies in Primary Hyperoxaluria Type I. BioMed Research International،Vol. 2013, no. 2013, pp.1-15.
https://search.emarefa.net/detail/BIM-1004867
Modern Language Association (MLA)
Pey, Angel L.…[et al.]. Protein Homeostasis Defects of Alanine-Glyoxylate Aminotransferase: New Therapeutic Strategies in Primary Hyperoxaluria Type I. BioMed Research International No. 2013 (2013), pp.1-15.
https://search.emarefa.net/detail/BIM-1004867
American Medical Association (AMA)
Pey, Angel L.& Albert, Armando& Salido, Eduardo. Protein Homeostasis Defects of Alanine-Glyoxylate Aminotransferase: New Therapeutic Strategies in Primary Hyperoxaluria Type I. BioMed Research International. 2013. Vol. 2013, no. 2013, pp.1-15.
https://search.emarefa.net/detail/BIM-1004867
Data Type
Journal Articles
Language
English
Notes
Includes bibliographical references
Record ID
BIM-1004867