Literature DB >> 33586075

Huntington's Disease: New Frontiers in Therapeutics.

Ling Pan1, Andrew Feigin2.   

Abstract

PURPOSE OF REVIEW: This article describes and discusses new potential disease-modifying therapies for Huntington's disease that are currently in human clinical trials as well as promising new therapies in preclinical development. RECENT
FINDINGS: Multiple potential disease-modifying therapeutics for HD are in active development, including direct DNA/gene therapies, RNA modulation, and therapies targeted at aberrant downstream pathways. The etiology of Huntington's disease (HD) is well-known as an abnormally expanded trinucleotide repeat within the huntingtin gene. However, the pathogenesis downstream of the mutant huntingtin gene is complex, involving multiple toxic pathways, including abnormal protein fragmentation and neuroinflammation. The current treatment of HD focuses largely on symptomatic management. This article discusses new, potential disease-modifying therapies that are currently in human clinical trials and preclinical development.

Entities:  

Keywords:  Clinical trials; Disease-modifying therapy; Gene therapy; Huntington’s disease; RNA interference

Year:  2021        PMID: 33586075     DOI: 10.1007/s11910-021-01093-3

Source DB:  PubMed          Journal:  Curr Neurol Neurosci Rep        ISSN: 1528-4042            Impact factor:   5.081


  57 in total

1.  Reversal of neuropathology and motor dysfunction in a conditional model of Huntington's disease.

Authors:  A Yamamoto; J J Lucas; R Hen
Journal:  Cell       Date:  2000-03-31       Impact factor: 41.582

Review 2.  Huntingtin aggregation and toxicity in Huntington's disease.

Authors:  Gillian Bates
Journal:  Lancet       Date:  2003-05-10       Impact factor: 79.321

Review 3.  Therapeutic approaches to Huntington disease: from the bench to the clinic.

Authors:  Nicholas S Caron; E Ray Dorsey; Michael R Hayden
Journal:  Nat Rev Drug Discov       Date:  2018-09-21       Impact factor: 84.694

4.  Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain.

Authors:  M DiFiglia; E Sapp; K O Chase; S W Davies; G P Bates; J P Vonsattel; N Aronin
Journal:  Science       Date:  1997-09-26       Impact factor: 47.728

5.  Altered brain neurotransmitter receptors in transgenic mice expressing a portion of an abnormal human huntington disease gene.

Authors:  J H Cha; C M Kosinski; J A Kerner; S A Alsdorf; L Mangiarini; S W Davies; J B Penney; G P Bates; A B Young
Journal:  Proc Natl Acad Sci U S A       Date:  1998-05-26       Impact factor: 11.205

Review 6.  Medical, Surgical, and Genetic Treatment of Huntington Disease.

Authors:  Christine M Stahl; Andrew Feigin
Journal:  Neurol Clin       Date:  2020-03-09       Impact factor: 3.806

7.  Exon 1 of the HD gene with an expanded CAG repeat is sufficient to cause a progressive neurological phenotype in transgenic mice.

Authors:  L Mangiarini; K Sathasivam; M Seller; B Cozens; A Harper; C Hetherington; M Lawton; Y Trottier; H Lehrach; S W Davies; G P Bates
Journal:  Cell       Date:  1996-11-01       Impact factor: 41.582

8.  Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation.

Authors:  S W Davies; M Turmaine; B A Cozens; M DiFiglia; A H Sharp; C A Ross; E Scherzinger; E E Wanker; L Mangiarini; G P Bates
Journal:  Cell       Date:  1997-08-08       Impact factor: 41.582

Review 9.  The incidence and prevalence of Huntington's disease: a systematic review and meta-analysis.

Authors:  Tamara Pringsheim; Katie Wiltshire; Lundy Day; Jonathan Dykeman; Thomas Steeves; Nathalie Jette
Journal:  Mov Disord       Date:  2012-06-12       Impact factor: 10.338

10.  Neuronal targets for reducing mutant huntingtin expression to ameliorate disease in a mouse model of Huntington's disease.

Authors:  Nan Wang; Michelle Gray; Xiao-Hong Lu; Jeffrey P Cantle; Sandra M Holley; Erin Greiner; Xiaofeng Gu; Dyna Shirasaki; Carlos Cepeda; Yuqing Li; Hongwei Dong; Michael S Levine; X William Yang
Journal:  Nat Med       Date:  2014-04-28       Impact factor: 53.440

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  4 in total

1.  A CAG repeat-targeting artificial miRNA lowers the mutant huntingtin level in the YAC128 model of Huntington's disease.

Authors:  Anna Kotowska-Zimmer; Lukasz Przybyl; Marianna Pewinska; Joanna Suszynska-Zajczyk; Dorota Wronka; Maciej Figiel; Marta Olejniczak
Journal:  Mol Ther Nucleic Acids       Date:  2022-05-05       Impact factor: 10.183

2.  Spectroscopic, Electrochemical, and Biological Assays of Copper-Binding Molecules for Screening of Different Drugs and Plant Extracts against Neurodegenerative Disorders.

Authors:  David Brinvillier; Melissa Barrast; Petra Couderc-Murillo; José Bono-Yagüe; Alain Rousteau; Ana Pilar Gómez Escribano; Marcos V Palmeira-Mello; Antonio Doménech-Carbó; Nady Passe-Coutrin; Muriel Sylvestre; Rafael P Vázquez-Manrique; Gerardo Cebrián-Torrejón
Journal:  ACS Omega       Date:  2022-05-04

3.  Anti-Huntington's Effect of Rosiridin via Oxidative Stress/AchE Inhibition and Modulation of Succinate Dehydrogenase, Nitrite, and BDNF Levels against 3-Nitropropionic Acid in Rodents.

Authors:  Muhammad Afzal; Nadeem Sayyed; Khalid Saad Alharbi; Sami I Alzarea; Mohammed Salem Alshammari; Fadhel A Alomar; Sattam Khulaif Alenezi; Anwarulabedin Mohsin Quazi; Abdulaziz I Alzarea; Imran Kazmi
Journal:  Biomolecules       Date:  2022-07-23

Review 4.  Nose-to-Brain: The Next Step for Stem Cell and Biomaterial Therapy in Neurological Disorders.

Authors:  Natalia Villar-Gómez; Doddy Denise Ojeda-Hernandez; Eneritz López-Muguruza; Silvia García-Flores; Natalia Bonel-García; María Soledad Benito-Martín; Belen Selma-Calvo; Alejandro Arturo Canales-Aguirre; Juan Carlos Mateos-Díaz; Paloma Montero-Escribano; Jordi A Matias-Guiu; Jorge Matías-Guiu; Ulises Gómez-Pinedo
Journal:  Cells       Date:  2022-10-01       Impact factor: 7.666

  4 in total

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