Matches in SemOpenAlex for { <https://semopenalex.org/work/W3187227029> ?p ?o ?g. }
- W3187227029 endingPage "948" @default.
- W3187227029 startingPage "948" @default.
- W3187227029 abstract "Mutations in Cu/Zn Superoxide Dismutase (SOD1) gene represent one of the most common causes of amyotrophic lateral sclerosis (ALS), a fatal neurodegenerative disorder that specifically affects motor neurons (MNs). The dismutase-active SOD1 G93A mutant is responsible for the formation of toxic aggregates onto the mitochondrial surface, using the Voltage-Dependent Anion Channel 1 (VDAC1) as an anchor point to the organelle. VDAC1 is the master regulator of cellular bioenergetics and by binding to hexokinases (HKs) it controls apoptosis. In ALS, however, SOD1 G93A impairs VDAC1 activity and displaces HK1 from mitochondria, promoting organelle dysfunction, and cell death. Using an ALS cell model, we demonstrate that a small synthetic peptide derived from the HK1 sequence (NHK1) recovers the cell viability in a dose–response manner and the defective mitochondrial respiration profile relative to the ADP phosphorylation. This correlates with an unexpected increase of VDAC1 expression and a reduction of SOD1 mutant accumulation at the mitochondrial level. Overall, our findings provide important new insights into the development of therapeutic molecules to fight ALS and help to better define the link between altered mitochondrial metabolism and MNs death in the disease." @default.
- W3187227029 created "2021-08-16" @default.
- W3187227029 creator A5025362782 @default.
- W3187227029 creator A5039074744 @default.
- W3187227029 creator A5040172058 @default.
- W3187227029 creator A5042513487 @default.
- W3187227029 creator A5060274257 @default.
- W3187227029 creator A5061631234 @default.
- W3187227029 creator A5061688720 @default.
- W3187227029 creator A5067261411 @default.
- W3187227029 creator A5076863636 @default.
- W3187227029 creator A5091693991 @default.
- W3187227029 date "2021-08-03" @default.
- W3187227029 modified "2023-10-16" @default.
- W3187227029 title "Small Hexokinase 1 Peptide against Toxic SOD1 G93A Mitochondrial Accumulation in ALS Rescues the ATP-Related Respiration" @default.
- W3187227029 cites W1644794975 @default.
- W3187227029 cites W1852530796 @default.
- W3187227029 cites W191921287 @default.
- W3187227029 cites W1978974783 @default.
- W3187227029 cites W1983990793 @default.
- W3187227029 cites W1986199954 @default.
- W3187227029 cites W1987998017 @default.
- W3187227029 cites W1999030622 @default.
- W3187227029 cites W1999899839 @default.
- W3187227029 cites W2009081910 @default.
- W3187227029 cites W2009866554 @default.
- W3187227029 cites W2012237324 @default.
- W3187227029 cites W2013135033 @default.
- W3187227029 cites W2015266340 @default.
- W3187227029 cites W2025629415 @default.
- W3187227029 cites W2025961979 @default.
- W3187227029 cites W2033564140 @default.
- W3187227029 cites W2036757369 @default.
- W3187227029 cites W2038697027 @default.
- W3187227029 cites W2050280746 @default.
- W3187227029 cites W2052238208 @default.
- W3187227029 cites W2054645758 @default.
- W3187227029 cites W2055905702 @default.
- W3187227029 cites W2065283382 @default.
- W3187227029 cites W2071591138 @default.
- W3187227029 cites W2075189940 @default.
- W3187227029 cites W2076386951 @default.
- W3187227029 cites W2077962324 @default.
- W3187227029 cites W2078040811 @default.
- W3187227029 cites W2079457709 @default.
- W3187227029 cites W2096386031 @default.
- W3187227029 cites W2096880567 @default.
- W3187227029 cites W2100262552 @default.
- W3187227029 cites W2107277218 @default.
- W3187227029 cites W2107489808 @default.
- W3187227029 cites W2107672209 @default.
- W3187227029 cites W2107798346 @default.
- W3187227029 cites W2115924220 @default.
- W3187227029 cites W2125258716 @default.
- W3187227029 cites W2127718436 @default.
- W3187227029 cites W2129141040 @default.
- W3187227029 cites W2133836078 @default.
- W3187227029 cites W2134087185 @default.
- W3187227029 cites W2139200038 @default.
- W3187227029 cites W2140387557 @default.
- W3187227029 cites W2144956444 @default.
- W3187227029 cites W2162033191 @default.
- W3187227029 cites W2162746575 @default.
- W3187227029 cites W2170666584 @default.
- W3187227029 cites W2191212519 @default.
- W3187227029 cites W2235176326 @default.
- W3187227029 cites W2236703262 @default.
- W3187227029 cites W2289080335 @default.
- W3187227029 cites W2307972733 @default.
- W3187227029 cites W2321985754 @default.
- W3187227029 cites W2530449264 @default.
- W3187227029 cites W2552888741 @default.
- W3187227029 cites W2566102821 @default.
- W3187227029 cites W2617427268 @default.
- W3187227029 cites W2620966663 @default.
- W3187227029 cites W2697643565 @default.
- W3187227029 cites W2776678223 @default.
- W3187227029 cites W2793061035 @default.
- W3187227029 cites W2796949439 @default.
- W3187227029 cites W2802074965 @default.
- W3187227029 cites W2806291212 @default.
- W3187227029 cites W2904950463 @default.
- W3187227029 cites W2912696061 @default.
- W3187227029 cites W2945168319 @default.
- W3187227029 cites W2957531246 @default.
- W3187227029 cites W2980841794 @default.
- W3187227029 cites W2982142319 @default.
- W3187227029 cites W2984250052 @default.
- W3187227029 cites W2999470740 @default.
- W3187227029 cites W3000958327 @default.
- W3187227029 cites W3038958603 @default.
- W3187227029 cites W3049263973 @default.
- W3187227029 cites W3092608235 @default.
- W3187227029 cites W3093928770 @default.
- W3187227029 cites W31051610 @default.
- W3187227029 cites W3109723868 @default.
- W3187227029 cites W3119091315 @default.
- W3187227029 cites W3119142493 @default.