Literature DB >> 28869595

A toxic mutant huntingtin species is resistant to selective autophagy.

Yuhua Fu1, Peng Wu1, Yuyin Pan1, Xiaoli Sun1,2, Huiya Yang1, Marian Difiglia3, Boxun Lu1,4.   

Abstract

Protein misfolding is a common theme in neurodegenerative disorders including Huntington's disease (HD). The HD-causing mutant huntingtin protein (mHTT) has an expanded polyglutamine (polyQ) stretch that may adopt multiple conformations, and the most toxic of these is the one recognized by antibody 3B5H10. Here we show that the 3B5H10-recognized mHTT species has a slower degradation rate due to its resistance to selective autophagy in human cells and brains, revealing mechanisms of its higher toxicity.

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Year:  2017        PMID: 28869595     DOI: 10.1038/nchembio.2461

Source DB:  PubMed          Journal:  Nat Chem Biol        ISSN: 1552-4450            Impact factor:   15.040


  33 in total

Review 1.  Selective autophagy mediated by autophagic adapter proteins.

Authors:  Terje Johansen; Trond Lamark
Journal:  Autophagy       Date:  2011-03       Impact factor: 16.016

2.  Single-step detection of mutant huntingtin in animal and human tissues: a bioassay for Huntington's disease.

Authors:  Andreas Weiss; Dorothée Abramowski; Miriam Bibel; Ruth Bodner; Vanita Chopra; Marian DiFiglia; Jonathan Fox; Kimberly Kegel; Corinna Klein; Stephan Grueninger; Steven Hersch; David Housman; Etienne Régulier; H Diana Rosas; Muriel Stefani; Scott Zeitlin; Graeme Bilbe; Paolo Paganetti
Journal:  Anal Biochem       Date:  2009-08-06       Impact factor: 3.365

Review 3.  Small changes, big impact: posttranslational modifications and function of huntingtin in Huntington disease.

Authors:  Dagmar E Ehrnhoefer; Liza Sutton; Michael R Hayden
Journal:  Neuroscientist       Date:  2011-02-10       Impact factor: 7.519

4.  Native mutant huntingtin in human brain: evidence for prevalence of full-length monomer.

Authors:  Ellen Sapp; Antonio Valencia; Xueyi Li; Neil Aronin; Kimberly B Kegel; Jean-Paul Vonsattel; Anne B Young; Nancy Wexler; Marian DiFiglia
Journal:  J Biol Chem       Date:  2012-02-27       Impact factor: 5.157

5.  CAG expansion affects the expression of mutant Huntingtin in the Huntington's disease brain.

Authors:  N Aronin; K Chase; C Young; E Sapp; C Schwarz; N Matta; R Kornreich; B Landwehrmeyer; E Bird; M F Beal
Journal:  Neuron       Date:  1995-11       Impact factor: 17.173

6.  Kinase inhibitors modulate huntingtin cell localization and toxicity.

Authors:  Randy Singh Atwal; Carly R Desmond; Nicholas Caron; Tamara Maiuri; Jianrun Xia; Simonetta Sipione; Ray Truant
Journal:  Nat Chem Biol       Date:  2011-05-29       Impact factor: 15.040

7.  Time course of early motor and neuropathological anomalies in a knock-in mouse model of Huntington's disease with 140 CAG repeats.

Authors:  Liliana B Menalled; Jessica D Sison; Ioannis Dragatsis; Scott Zeitlin; Marie-Françoise Chesselet
Journal:  J Comp Neurol       Date:  2003-10-06       Impact factor: 3.215

8.  p62/SQSTM1 binds directly to Atg8/LC3 to facilitate degradation of ubiquitinated protein aggregates by autophagy.

Authors:  Serhiy Pankiv; Terje Høyvarde Clausen; Trond Lamark; Andreas Brech; Jack-Ansgar Bruun; Heidi Outzen; Aud Øvervatn; Geir Bjørkøy; Terje Johansen
Journal:  J Biol Chem       Date:  2007-06-19       Impact factor: 5.157

9.  Identifying polyglutamine protein species in situ that best predict neurodegeneration.

Authors:  Jason Miller; Montserrat Arrasate; Elizabeth Brooks; Clare Peters Libeu; Justin Legleiter; Danny Hatters; Jessica Curtis; Kenneth Cheung; Preethi Krishnan; Siddhartha Mitra; Kartika Widjaja; Benjamin A Shaby; Gregor P Lotz; Yvonne Newhouse; Emily J Mitchell; Alex Osmand; Michelle Gray; Vanitha Thulasiramin; Frédéric Saudou; Mark Segal; X William Yang; Eliezer Masliah; Leslie M Thompson; Paul J Muchowski; Karl H Weisgraber; Steven Finkbeiner
Journal:  Nat Chem Biol       Date:  2011-10-30       Impact factor: 15.040

10.  A striatal-enriched intronic GPCR modulates huntingtin levels and toxicity.

Authors:  Yuwei Yao; Xiaotian Cui; Ismael Al-Ramahi; Xiaoli Sun; Bo Li; Jiapeng Hou; Marian Difiglia; James Palacino; Zhi-Ying Wu; Lixiang Ma; Juan Botas; Boxun Lu
Journal:  Elife       Date:  2015-03-04       Impact factor: 8.140

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  14 in total

1.  Mutant huntingtin inhibits the mitochondrial unfolded protein response by impairing ABCB10 mRNA stability.

Authors:  Zixing Fu; Fang Liu; Chunyue Liu; Beifang Jin; Yueqing Jiang; Mingliang Tang; Xin Qi; Xing Guo
Journal:  Biochim Biophys Acta Mol Basis Dis       Date:  2019-02-23       Impact factor: 5.187

Review 2.  Proteostasis in Huntington's disease: disease mechanisms and therapeutic opportunities.

Authors:  Rachel J Harding; Yu-Feng Tong
Journal:  Acta Pharmacol Sin       Date:  2018-04-05       Impact factor: 6.150

Review 3.  Emerging New Concepts of Degrader Technologies.

Authors:  Yu Ding; Yiyan Fei; Boxun Lu
Journal:  Trends Pharmacol Sci       Date:  2020-04-23       Impact factor: 14.819

Review 4.  Autophagy in health and disease: From molecular mechanisms to therapeutic target.

Authors:  Guang Lu; Yu Wang; Yin Shi; Zhe Zhang; Canhua Huang; Weifeng He; Chuang Wang; Han-Ming Shen
Journal:  MedComm (2020)       Date:  2022-07-10

5.  Restoration of CTSD (cathepsin D) and lysosomal function in stroke is neuroprotective.

Authors:  M Iqbal Hossain; Joshua M Marcus; Jun Hee Lee; Patrick L Garcia; VinodKumar Singh; John J Shacka; Jianhua Zhang; Toby I Gropen; Charles N Falany; Shaida A Andrabi
Journal:  Autophagy       Date:  2020-05-25       Impact factor: 16.016

Review 6.  Association Between Autophagy and Neurodegenerative Diseases.

Authors:  Nobuhiro Fujikake; Minkyoung Shin; Shigeomi Shimizu
Journal:  Front Neurosci       Date:  2018-05-22       Impact factor: 4.677

Review 7.  Mechanisms of protein toxicity in neurodegenerative diseases.

Authors:  Chang Geon Chung; Hyosang Lee; Sung Bae Lee
Journal:  Cell Mol Life Sci       Date:  2018-06-12       Impact factor: 9.261

8.  Covalent targeting of the vacuolar H+-ATPase activates autophagy via mTORC1 inhibition.

Authors:  Clive Yik-Sham Chung; Hijai R Shin; Charles A Berdan; Breanna Ford; Carl C Ward; James A Olzmann; Roberto Zoncu; Daniel K Nomura
Journal:  Nat Chem Biol       Date:  2019-07-08       Impact factor: 15.040

Review 9.  Autophagy in Neurodegenerative Diseases: A Hunter for Aggregates.

Authors:  Hyungsun Park; Ju-Hee Kang; Seongju Lee
Journal:  Int J Mol Sci       Date:  2020-05-10       Impact factor: 5.923

Review 10.  Unconventional Secretion and Intercellular Transfer of Mutant Huntingtin.

Authors:  Bor Luen Tang
Journal:  Cells       Date:  2018-06-14       Impact factor: 6.600

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