Decreased intracellular ATP content and intact mitochondrial energy generating capacity in human cystinotic fibroblasts
about
Identification and subcellular localization of a new cystinosin isoformCystine accumulation attenuates insulin release from the pancreatic β-cell due to elevated oxidative stress and decreased ATP levels.Mitochondrial autophagy promotes cellular injury in nephropathic cystinosisAnalysis of CTNS gene transcripts in nephropathic cystinosisInhibition of intracellular clusterin attenuates cell death in nephropathic cystinosis.Treatment of corneal cystine crystal accumulation in patients with cystinosis.A mouse model suggests two mechanisms for thyroid alterations in infantile cystinosis: decreased thyroglobulin synthesis due to endoplasmic reticulum stress/unfolded protein response and impaired lysosomal processing.Mitochondrial energetics and therapeutics.Muscle wasting and adipose tissue browning in infantile nephropathic cystinosis.The pathogenesis of cystinosis: mechanisms beyond cystine accumulation.Lysosome dysfunction in the pathogenesis of kidney diseases.The renal Fanconi syndrome in cystinosis: pathogenic insights and therapeutic perspectives.Lysosomal cystine accumulation promotes mitochondrial depolarization and induction of redox-sensitive genes in human kidney proximal tubular cells.Cystine dimethylester loading promotes oxidative stress and a reduction in ATP independent of lysosomal cystine accumulation in a human proximal tubular epithelial cell line.p62/SQSTM1 prominently accumulates in renal proximal tubules in nephropathic cystinosis.Mitochondrial complex V expression and activity in cystinotic fibroblasts.Slow progression of renal failure in a child with infantile cystinosis.Nephropathic Cystinosis: Symptoms, Treatment, and Perspectives of a Systemic Disease.Evaluation of the proximal tubular function in hereditary renal Fanconi syndrome.Thiol/disulfide status regulates the activity of thiol-containing kinases related to energy homeostasis in rat kidney.
P2860
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P2860
Decreased intracellular ATP content and intact mitochondrial energy generating capacity in human cystinotic fibroblasts
description
2006 nî lūn-bûn
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2006 թուականի Փետրուարին հրատարակուած գիտական յօդուած
@hyw
2006 թվականի փետրվարին հրատարակված գիտական հոդված
@hy
2006年の論文
@ja
2006年論文
@yue
2006年論文
@zh-hant
2006年論文
@zh-hk
2006年論文
@zh-mo
2006年論文
@zh-tw
2006年论文
@wuu
name
Decreased intracellular ATP co ...... n human cystinotic fibroblasts
@ast
Decreased intracellular ATP co ...... n human cystinotic fibroblasts
@en
Decreased intracellular ATP co ...... n human cystinotic fibroblasts
@en-gb
Decreased intracellular ATP co ...... n human cystinotic fibroblasts
@nl
type
label
Decreased intracellular ATP co ...... n human cystinotic fibroblasts
@ast
Decreased intracellular ATP co ...... n human cystinotic fibroblasts
@en
Decreased intracellular ATP co ...... n human cystinotic fibroblasts
@en-gb
Decreased intracellular ATP co ...... n human cystinotic fibroblasts
@nl
prefLabel
Decreased intracellular ATP co ...... n human cystinotic fibroblasts
@ast
Decreased intracellular ATP co ...... n human cystinotic fibroblasts
@en
Decreased intracellular ATP co ...... n human cystinotic fibroblasts
@en-gb
Decreased intracellular ATP co ...... n human cystinotic fibroblasts
@nl
P2093
P2860
P50
P921
P1433
P1476
Decreased intracellular ATP co ...... n human cystinotic fibroblasts
@en
P2093
Adriana de Graaf-Hess
Elena N Levtchenko
Henk-Jan Visch
Jan B Koenderink
Lambertus P van den Heuvel
Leo A Monnens
Martijn J G Wilmer
P2860
P304
P356
10.1203/01.PDR.0000196334.46940.54
P407
P577
2006-02-01T00:00:00Z
P5875
P6179
1039537048