scholarly journals p53 increases caspase-6 expression and activation in muscle tissue expressing mutant huntingtin

2013 ◽  
Vol 23 (3) ◽  
pp. 717-729 ◽  
Author(s):  
Dagmar E. Ehrnhoefer ◽  
Niels H. Skotte ◽  
Safia Ladha ◽  
Yen T.N. Nguyen ◽  
Xiaofan Qiu ◽  
...  
2012 ◽  
Vol 32 (1) ◽  
pp. 183-193 ◽  
Author(s):  
E. Waldron-Roby ◽  
T. Ratovitski ◽  
X. Wang ◽  
M. Jiang ◽  
E. Watkin ◽  
...  

PLoS Currents ◽  
2012 ◽  
Vol 4 ◽  
pp. e4fd085bfc9973 ◽  
Author(s):  
Christian Landles ◽  
Andreas Weiss ◽  
Sophie Franklin ◽  
David Howland ◽  
Gill Bates

Cell ◽  
2006 ◽  
Vol 125 (6) ◽  
pp. 1179-1191 ◽  
Author(s):  
Rona K. Graham ◽  
Yu Deng ◽  
Elizabeth J. Slow ◽  
Brendan Haigh ◽  
Nagat Bissada ◽  
...  

2021 ◽  
Author(s):  
Abdullah Aslan ◽  
Muhammed Ismail Can ◽  
Ozlem Gok ◽  
Seda Beyaz ◽  
Gozde Parlak ◽  
...  

Abstract In this study, 42 Wistar albino female rats (n = 42, 8 weeks old) were used. Rats were divided into 6 groups and 7 rats included each group. Groups: (i) Control Group: Standard diet; (ii) RJ (royal jelly) Group: Standard diet + royal jelly; (iii) F50 Group: Standard diet + 50 mg/kg fluoride; (iv): F100 Group: Standard diet + 100 mg/kg fluoride; (v) F50 + RJ Group: Standard diet + 50 mg/kg fluoride + royal jelly; (iv): F100 + RJ Group: Standard diet + 100 mg/kg fluoride + royal jelly. After the 8-week study period, the rats were decapitated and their muscle tissues were removed. Expression levels of Caspase-3, Caspase-6, Bax, Tnf-α, IL1-α and Bcl-2 proteins in muscle tissue were determined by Western Blotting method. Histopathological analyzes were also performed on the muscle tissue. MDA, GSH, and CAT analyzes were determined by spectrophotometric analysis. According to our findings, Bcl-2, Tnf-α and IL1-α protein expression were increased in damage groups compared to control and royal jelly groups, Caspase-3, Caspase-6 and Bax protein expression levels decreased in damage groups. There was an increase in MDA level in damage groups compared to the control and royal jelly groups, CAT and GSH levels decreased in damage groups. According to histopathological analysis results, edema and inflammatory cell formations were found in the injury groups, a tendency to decrease in these injuries was observed in the treatment groups. Based on these results, we can say that royal jelly has protective effects against fluoride damage.


2012 ◽  
Vol 1 (2) ◽  
pp. 243-260 ◽  
Author(s):  
Rona K. Graham ◽  
Yu Deng ◽  
Mahmoud A. Pouladi ◽  
Kuljeet Vaid ◽  
Dagmar Ehrnhoefer ◽  
...  

2017 ◽  
Author(s):  
D.D.O. Martin ◽  
M. E. Schmidt ◽  
Y. T. Nguyen ◽  
N. Lazic ◽  
M. R. Hayden

ABSTRACTHuntington disease (HD) is a progressive neurodegenerative disease that initially affects the striatum leading to changes in behavior and loss of motor coordination. It is caused by an expansion in the polyglutamine repeat at the N-terminus of huntingtin (HTT) that leads to aggregation of mutant HTT. The loss of wildtype function, in combination with the toxic gain of function mutation, initiates various cell death pathways. Wildtype and mutant HTT are regulated by different post-translational modifications that can positively or negatively regulate their function or toxicity. In particular, we have previously shown that caspase cleavage of mutant HTT at amino acid position aspartate 586 (D586) by caspase-6 is critical for the pathogenesis of the disease in an HD mouse model. Herein, we describe the identification of a new caspase cleavage site at position D572 that is mediated by caspase-1. Inhibition of caspase-1 also inhibits cleavage at D586 through inhibition of caspase-6. Inhibition of caspase cleavage at D572 significantly decreases mutant HTT aggregation and significantly increased the turnover of soluble mutant HTT. This suggests that caspase-1 may be a viable target to inhibit caspase cleavage of mutant HTT at both D572 and D586 to promote mutant HTT clearance.


2010 ◽  
Vol 30 (45) ◽  
pp. 15019-15029 ◽  
Author(s):  
R. K. Graham ◽  
Y. Deng ◽  
J. Carroll ◽  
K. Vaid ◽  
C. Cowan ◽  
...  

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