Targeting proximal tubule mitochondrial dysfunction attenuates the renal disease of methylmalonic acidemia

Proceedings of the National Academy of Sciences of the United States of America

Volumn 110, Issue 33, 2013, Pages 13552-13557

  Manoli, Irini ^a   Sysol, Justin R ^a   Li, Lingli ^b   Houillier, Pascal ^b,c   Garone, Caterina ^d,e   Wang, Cindy ^a   Zerfas, Patricia M ^f   Cusmano Ozog, Kristina ^g   Young, Sarah ^h   Trivedi, Niraj S ^a   Cheng, Jun ^a   Sloan, Jennifer L ^a   Chandler, Randy J ^a   Abu Asab, Mones ⁱ   Tsokos, Maria ⁱ   Elkahloun, Abdel G ^a   Rosen, Seymour ^j,k   Enns, Gregory M ^l   Berry, Gerard T ^l   Hoffmann, Victoria ^f   DiMauro, Salvatore ^d   Schnermann, Jurgen ^b   Venditti, Charles P ^a,b   more..

^a NATIONAL HUMAN GENOME RESEARCH INSTITUTE   (United States)

^b NATIONAL INSTITUTE OF DIABETES AND DIGESTIVE AND KIDNEY DISEASES   (United States)

^c INSERM   (France)

^d Columbia University ^*  (United States)

^e UNIVERSITY OF BOLOGNA   (Italy)

^f NATIONAL INSTITUTES OF HEALTH   (United States)

^g STANFORD UNIVERSITY   (United States)

^h DUKE UNIVERSITY MEDICAL CENTER   (United States)

ⁱ NATIONAL CANCER INSTITUTE   (United States)

^j BETH ISRAEL DEACONESS MEDICAL CENTER   (United States)

^k NONE   (United States)

^l BOSTON CHILDREN S HOSPITAL   (United States)

more..

Author keywords

Chronic renal failure; Cobalamin; Megamitochondria; Organic acidemia

Indexed keywords

ALBUMIN; BIOLOGICAL MARKER; MUT PROTEIN; NEUTROPHIL GELATINASE ASSOCIATED LIPOCALIN; UNCLASSIFIED DRUG;

ANIMAL EXPERIMENT; ANIMAL MODEL; ARTICLE; COHORT ANALYSIS; CONTROLLED STUDY; GLOMERULUS FILTRATION RATE; INTERSTITIAL NEPHRITIS; KIDNEY BIOPSY; KIDNEY DISEASE; KIDNEY DYSFUNCTION; KIDNEY PROXIMAL TUBULE; KIDNEY TUBULE ABSORPTION; KIDNEY TUBULE FUNCTION; LETHALITY; MALE; METHYLMALONIC ACIDEMIA; MICROARRAY ANALYSIS; MITOCHONDRION; MOUSE; NEPHRON; NONHUMAN; OXIDATIVE STRESS; PRIORITY JOURNAL; PROMOTER REGION; PROTEIN EXPRESSION; TRANSGENE;

CHRONIC RENAL FAILURE; COBALAMIN; MEGAMITOCHONDRIA; ORGANIC ACIDEMIA;

AMINO ACID METABOLISM, INBORN ERRORS; ANIMALS; ANTIOXIDANTS; BIOLOGICAL MARKERS; BLOTTING, WESTERN; DISEASE MODELS, ANIMAL; DNA PRIMERS; ENZYME-LINKED IMMUNOSORBENT ASSAY; FLUORESCEIN-5-ISOTHIOCYANATE; GENOTYPE; GLOMERULAR FILTRATION RATE; HUMANS; IMMUNOHISTOCHEMISTRY; KIDNEY TUBULES, PROXIMAL; METHYLMALONYL-COA MUTASE; MICE; MICE, KNOCKOUT; MICROARRAY ANALYSIS; MICROSCOPY, ELECTRON, TRANSMISSION; NEPHRITIS, INTERSTITIAL; REAL-TIME POLYMERASE CHAIN REACTION; TRANSGENES; UBIQUINONE;

EID: 84882415756     PISSN: 00278424     EISSN: 10916490     Source Type: Journal    
DOI: 10.1073/pnas.1302764110     Document Type: Article

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