Targeting proximal tubule mitochondrial dysfunction attenuates the renal disease of methylmalonic ac...
Targeting proximal tubule mitochondrial dysfunction attenuates the renal disease of methylmalonic acidemia
About this item
Full title
Author / Creator
Manoli, Irini , Sysol, Justin R. , Li, Lingli , Houillier, Pascal , Garone, Caterina , Wang, Cindy , Zerfas, Patricia M. , Cusmano-Ozog, Kristina , Young, Sarah , Trivedi, Niraj S. , Cheng, Jun , Sloan, Jennifer L. , Chandler, Randy J. , Abu-Asab, Mones , Tsokos, Maria , Elkahloun, Abdel G. , Rosen, Seymour , Enns, Gregory M. , Berry, Gerard T. , Hoffmann, Victoria , DiMauro, Salvatore , Schnermann, Jurgen and Venditti, Charles P.
Publisher
United States: National Academy of Sciences
Journal title
Language
English
Formats
Publication information
Publisher
United States: National Academy of Sciences
Subjects
More information
Scope and Contents
Contents
Isolated methylmalonic acidemia (MMA), caused by deficiency of the mitochondrial enzyme methylmalonyl-CoA mutase (MUT), is often complicated by end stage renal disease that is resistant to conventional therapies, including liver transplantation. To establish a viable model of MMA renal disease, Mut was expressed in the liver of Mut ⁻/⁻ mice as a st...
Alternative Titles
Full title
Targeting proximal tubule mitochondrial dysfunction attenuates the renal disease of methylmalonic acidemia
Authors, Artists and Contributors
Author / Creator
Sysol, Justin R.
Li, Lingli
Houillier, Pascal
Garone, Caterina
Wang, Cindy
Zerfas, Patricia M.
Cusmano-Ozog, Kristina
Young, Sarah
Trivedi, Niraj S.
Cheng, Jun
Sloan, Jennifer L.
Chandler, Randy J.
Abu-Asab, Mones
Tsokos, Maria
Elkahloun, Abdel G.
Rosen, Seymour
Enns, Gregory M.
Berry, Gerard T.
Hoffmann, Victoria
DiMauro, Salvatore
Schnermann, Jurgen
Venditti, Charles P.
Identifiers
Primary Identifiers
Record Identifier
TN_cdi_jstor_primary_42712945
Permalink
https://devfeature-collection.sl.nsw.gov.au/record/TN_cdi_jstor_primary_42712945
Other Identifiers
ISSN
0027-8424,1091-6490
E-ISSN
1091-6490
DOI
10.1073/pnas.1302764110
