TY  - JOUR
AU  - Kleiner, Giulio
AU  - Barca, Emanuele
AU  - Ziosi, Marcello
AU  - Emmanuele, Valentina
AU  - Xu, Yimeng
AU  - Hidalgo-Gutierrez, Agustin
AU  - Qiao, Changhong
AU  - Tadesse, Saba
AU  - Area-Gomez, Estela
AU  - Lopez, Luis C
AU  - Quinzii, Catarina M
PY  - 2018
DO  - 10.1016/j.bbadis.2018.09.002
UR  - http://hdl.handle.net/10668/12994
T2  - Biochimica et biophysica acta. Molecular basis of disease
AB  - Nephrotic syndrome (NS), a frequent chronic kidney disease in children and young adults, is the most common phenotype associated with primary coenzyme Q10 (CoQ10) deficiency and is very responsive to CoQ10 supplementation, although the pathomechanism...
LA  - en
KW  - CoQ deficiency
KW  - Coenzyme Q(10)
KW  - Mitochondria
KW  - Oxidative stress
KW  - Sulfides
KW  - Alkyl and Aryl Transferases
KW  - Animals
KW  - Antioxidants
KW  - Ataxia
KW  - Disease Models, Animal
KW  - HeLa Cells
KW  - Humans
KW  - Hydrogen Sulfide
KW  - Kidney
KW  - Metabolic Networks and Pathways
KW  - Mice
KW  - Mice, Transgenic
KW  - Mitochondria
KW  - Mitochondrial Diseases
KW  - Muscle Weakness
KW  - Nephrotic Syndrome
KW  - Oxidation-Reduction
KW  - Oxidative Stress
KW  - Oxidoreductases Acting on Sulfur Group Donors
KW  - Reactive Oxygen Species
KW  - Ubiquinone
TI  - CoQ10 supplementation rescues nephrotic syndrome through normalization of H2S oxidation pathway.
TY  - research article
VL  - 1864
ER  -