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PMID |
Sentence |
1 |
9714816
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In both these disorders, the mitochondrial abnormality is secondary to the primary nuclear mutation:CAG repeat in the huntingtin gene in HD, and GAA repeat in the frataxin gene in FA.
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2 |
12470650
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It was noteworthy that the Wnt-1 inducible signaling pathway protein-1 (WISP-1), Ras-associated protein 1B (Rap1B), vascular cell adhesion molecule-1 (VCAM-1), and huntingtin interacting protein genes (HIP) were observed to be over-expressed during pancreas regeneration.
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3 |
12586550
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Pancreatic islets from HD transgenic mice express reduced levels of the pancreatic islet hormones insulin, somatostatin, and glucagon and exhibit intrinsic defects in insulin production.
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4 |
12586550
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HD transgenic mice develop an age-dependent reduction of insulin mRNA expression and diminished expression of key regulators of insulin gene transcription, including the pancreatic homeoprotein PDX-1, E2A proteins, and the coactivators CBP and p300.
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5 |
12586550
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Disrupted expression of a subset of transcription factors in pancreatic beta cells by a polyglutamine expansion tract in the huntingtin protein selectively impairs insulin gene expression to result in insulin deficiency and diabetes.
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6 |
18640979
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Rosiglitazone treatment prevents mitochondrial dysfunction in mutant huntingtin-expressing cells: possible role of peroxisome proliferator-activated receptor-gamma (PPARgamma) in the pathogenesis of Huntington disease.
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7 |
18640979
|
Peroxisome proliferator-activated receptor-gamma (PPARgamma) is a member of the PPAR family of transcription factors.
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8 |
18640979
|
Synthetic PPARgamma agonists are used as oral anti-hyperglycemic drugs for the treatment of non-insulin-dependent diabetes.
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9 |
18640979
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Altogether, this evidence indicates that PPARgamma activation by rosiglitazone attenuates mitochondrial dysfunction in mutant huntingtin-expressing striatal cells, and this could be an important therapeutic avenue to ameliorate the mitochondrial dysfunction that occurs in HD.
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10 |
18640979
|
Rosiglitazone treatment prevents mitochondrial dysfunction in mutant huntingtin-expressing cells: possible role of peroxisome proliferator-activated receptor-gamma (PPARgamma) in the pathogenesis of Huntington disease.
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11 |
18640979
|
Peroxisome proliferator-activated receptor-gamma (PPARgamma) is a member of the PPAR family of transcription factors.
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12 |
18640979
|
Synthetic PPARgamma agonists are used as oral anti-hyperglycemic drugs for the treatment of non-insulin-dependent diabetes.
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13 |
18640979
|
Altogether, this evidence indicates that PPARgamma activation by rosiglitazone attenuates mitochondrial dysfunction in mutant huntingtin-expressing striatal cells, and this could be an important therapeutic avenue to ameliorate the mitochondrial dysfunction that occurs in HD.
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14 |
19628478
|
Mutant huntingtin interacts with {beta}-tubulin and disrupts vesicular transport and insulin secretion.
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15 |
19628478
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Here, we show that mutant huntingtin disrupts intracellular transport and insulin secretion by direct interference with microtubular beta-tubulin.
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16 |
19628478
|
We demonstrate that mutant huntingtin impairs glucose-stimulated insulin secretion in insulin-producing beta-cells, without altering stored levels of insulin.
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17 |
19628478
|
Mutant huntingtin interacts with {beta}-tubulin and disrupts vesicular transport and insulin secretion.
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18 |
19628478
|
Here, we show that mutant huntingtin disrupts intracellular transport and insulin secretion by direct interference with microtubular beta-tubulin.
|
19 |
19628478
|
We demonstrate that mutant huntingtin impairs glucose-stimulated insulin secretion in insulin-producing beta-cells, without altering stored levels of insulin.
|
20 |
19628478
|
Mutant huntingtin interacts with {beta}-tubulin and disrupts vesicular transport and insulin secretion.
|
21 |
19628478
|
Here, we show that mutant huntingtin disrupts intracellular transport and insulin secretion by direct interference with microtubular beta-tubulin.
|
22 |
19628478
|
We demonstrate that mutant huntingtin impairs glucose-stimulated insulin secretion in insulin-producing beta-cells, without altering stored levels of insulin.
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23 |
19661690
|
HSP40 ameliorates impairment of insulin secretion by inhibiting huntingtin aggregation in a HD pancreatic beta cell model.
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24 |
19661690
|
Here, we found that mutant huntingtin forms aggregates in the cytoplasm and reduces insulin secretion from huntingtin transfected pancreatic beta cell lines, NIT-1 cells.
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25 |
19661690
|
Activity of the pro-survival factor, Akt, is enhanced in these cells, which might improve the maintenance of insulin content.
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26 |
19661690
|
Overexpression of heat shock protein 40 (HSP40) inhibits aggregation, reverses impaired insulin release, and blocks the enhancement of Akt activity.
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27 |
19661690
|
These results suggest that impairment of beta cells is mostly linked with the aggregate formation of mutant huntingtin, and that HSP40 ameliorates the malfunction of pancreatic beta cells by inhibiting aggregation.
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28 |
19661690
|
HSP40 ameliorates impairment of insulin secretion by inhibiting huntingtin aggregation in a HD pancreatic beta cell model.
|
29 |
19661690
|
Here, we found that mutant huntingtin forms aggregates in the cytoplasm and reduces insulin secretion from huntingtin transfected pancreatic beta cell lines, NIT-1 cells.
|
30 |
19661690
|
Activity of the pro-survival factor, Akt, is enhanced in these cells, which might improve the maintenance of insulin content.
|
31 |
19661690
|
Overexpression of heat shock protein 40 (HSP40) inhibits aggregation, reverses impaired insulin release, and blocks the enhancement of Akt activity.
|
32 |
19661690
|
These results suggest that impairment of beta cells is mostly linked with the aggregate formation of mutant huntingtin, and that HSP40 ameliorates the malfunction of pancreatic beta cells by inhibiting aggregation.
|
33 |
19661690
|
HSP40 ameliorates impairment of insulin secretion by inhibiting huntingtin aggregation in a HD pancreatic beta cell model.
|
34 |
19661690
|
Here, we found that mutant huntingtin forms aggregates in the cytoplasm and reduces insulin secretion from huntingtin transfected pancreatic beta cell lines, NIT-1 cells.
|
35 |
19661690
|
Activity of the pro-survival factor, Akt, is enhanced in these cells, which might improve the maintenance of insulin content.
|
36 |
19661690
|
Overexpression of heat shock protein 40 (HSP40) inhibits aggregation, reverses impaired insulin release, and blocks the enhancement of Akt activity.
|
37 |
19661690
|
These results suggest that impairment of beta cells is mostly linked with the aggregate formation of mutant huntingtin, and that HSP40 ameliorates the malfunction of pancreatic beta cells by inhibiting aggregation.
|
38 |
21571086
|
Prion protein (Prion diseases), amyloid-beta (Alzheimer's disease), alpha-synuclein (Parkinson's disease), Huntingtin (Huntington's disease), serum amyloid A (AA amyloidosis) and islet amyloid polypeptide (type 2 diabetes) are some of the proteins that trigger disease when they get misfolded.
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39 |
21705657
|
Huntingtin-interacting protein 14 is a type 1 diabetes candidate protein regulating insulin secretion and beta-cell apoptosis.
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40 |
21705657
|
An unexpected top-scoring candidate gene was huntingtin-interacting protein (HIP)-14/ZDHHC17.
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41 |
21705657
|
Immunohistochemical analysis of pancreatic sections demonstrated that HIP14 is almost exclusively expressed in insulin-positive cells in islets of Langerhans.
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42 |
21705657
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RNAi knockdown experiments established that HIP14 is an antiapoptotic protein required for β-cell survival and glucose-stimulated insulin secretion.
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43 |
21705657
|
Proinflammatory cytokines (IL-1β and IFN-γ) that mediate β-cell dysfunction in T1D down-regulated HIP14 expression in insulin-secreting INS-1 cells and in isolated rat and human islets.
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44 |
21705657
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Overexpression of HIP14 was associated with a decrease in IL-1β-induced NF-κB activity and protection against IL-1β-mediated apoptosis.
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