7 documents found, page 1 of 1

Histone Deacetylase Inhibitors Protect Against Pyruvate Dehydrogenase Dysfuncti...

Transcriptional deregulation and changes in mitochondrial bioenergetics, including pyruvate dehydrogenase (PDH) dysfunction, have been described in Huntington's disease (HD). We showed previously that the histone deacetylase inhibitors (HDACIs) trichostatin A and sodium butyrate (SB) ameliorate mitochondrial function in cells expressing mutant huntingtin. In this work, we investigated the effect of HDACIs on th...

Ontology-based feature modeling: an empirical study in changing scenarios

Activation of IGF-1 and Insulin Signaling Pathways Ameliorate Mitochondrial Fun...

Huntington's disease (HD) is an inherited neurodegenerative disease caused by a polyglutamine repeat expansion in the huntingtin protein. Mitochondrial dysfunction associated with energy failure plays an important role in this untreated pathology. In the present work, we used lymphoblasts obtained from HD patients or unaffected parentally related individuals to study the protective role of insulin-like growth f...

Quantifying the effects of aspectual decompositions on design by contract modul...

Texto completo: acesso restrito. p. 913–941; Submitted by Suelen Reis (suziy.ellen@gmail.com) on 2014-05-08T12:00:48Z No. of bitstreams: 1 0218194013500265.pdf: 1599729 bytes, checksum: b3e54b45560b15d918aed9454ee5a3fc (MD5); Made available in DSpace on 2014-05-08T12:00:48Z (GMT). No. of bitstreams: 1 0218194013500265.pdf: 1599729 bytes, checksum: b3e54b45560b15d918aed9454ee5a3fc (MD5) Previous issue date: 2013...

Insulin and IGF-1 improve mitochondrial function in a PI-3K/Akt-dependent manne...

Akt, protein kinase B; ARE, antioxidant response element; Erk, extracellular signal-regulated kinase; CBP, CREB-binding protein; CREB, cAMP response-element (CRE) binding protein; CDK, cyclin-dependent kinase; DHE, dihydroethidium; Drp1, dynamin-related protein 1 or dynamin 1-like (DNM1L); GCL, glutamate-cysteine ligase; GCLc, glutamate-cysteine catalytic subunit; GPx, glutathione peroxidase; GSH, glutathione, ...

Glutathione redox cycle dysregulation in Huntington’s disease knock-in striatal...

Huntington’s disease (HD) is a CAG repeat disorder affecting the HD gene, which encodes for huntingtin (Htt) and is characterized by prominent cell death in the striatum. Oxidative stress was previously implicated in HD neurodegeneration, but the role of the major endogenous antioxidant system, the glutathione redox cycle, has been less studied following expression of full-length mutant Htt (FL-mHtt). Thus, in ...

Bioenergetic dysfunction in Huntington's disease human cybrids

In this work we studied the mitochondrial-associated metabolic pathways in Huntington's disease (HD) versus control (CTR) cybrids, a cell model in which the contribution of mitochondrial defects from patients is isolated. HD cybrids exhibited an interesting increase in ATP levels, when compared to CTR cybrids. Concomitantly, we observed increased glycolytic rate in HD cybrids, as revealed by increased lactate/p...