Review
Environmental Sciences
Sukhbir Singh, Hema, Neelam Sharma, Monika Sachdeva, Tapan Behl, Ishrat Zahoor, Neeraj Kumar Fuloria, Mahendran Sekar, Shivkanya Fuloria, Vetriselvan Subramaniyan, Amal M. Alsubayiel, Hamed Ghaleb Dailah, Tanveer Naved, Saurabh Bhatia, Ahmed Al-Harrasi, Lotfi Aleya
Summary: Neurodegeneration refers to the loss of neuronal capacity and structure over time, leading to neurodegenerative disorders such as Alzheimer's, amyotrophic lateral sclerosis, Parkinson's, and Huntington's disease (HD). This review primarily focuses on HD, a common single-gene neurological disorder. HD affects the entire body and brain, with a mutant huntingtin polyglutamine sequence that leads to apoptosis and dysfunction. Nanoparticles have emerged as a promising strategy for the treatment of HD, as they can cross the blood-brain barrier and reduce drug toxicity. This review provides an overview of HD, including its symptoms, genetics, and management, and discusses the latest studies on nanotechnology-related technologies for HD treatment.
ENVIRONMENTAL SCIENCE AND POLLUTION RESEARCH
(2022)
Article
Chemistry, Multidisciplinary
Alvaro Martinez-Camarena, Marian Merino, Ana Virginia Sanchez-Sanchez, Salvador Blasco, Jose M. Llinares, Jose L. Mullor, Enrique Garcia-Espana
Summary: A novel amino-nanozyme based on boehmite nanoparticles has been designed to address important issues in Huntington disease. The amino-nanozyme exhibits SOD activity, mitoROS scavenging properties, and the ability to disaggregate mutant huntingtin deposits in cells.
CHEMICAL COMMUNICATIONS
(2022)
Article
Biology
Sushila A. Shenoy, Sushuang Zheng, Wencheng Liu, Yuanyi Dai, Yuanxiu Liu, Zhipeng Hou, Susumu Mori, Yi Tang, Jerry Cheng, Wenzhen Duan, Chenjian Li
Summary: Here we report the successful generation and characterization of a novel Huntington's disease mouse model BAC226Q, which exhibits HD-like phenotypes and can serve as a valuable tool for studying disease mechanisms and testing therapeutic approaches.
Review
Biochemistry & Molecular Biology
Li Liu, Huichun Tong, Yize Sun, Xingxing Chen, Tianqi Yang, Gongke Zhou, Xiao-Jiang Li, Shihua Li
Summary: Huntington's disease (HD) is caused by an expansion of a CAG repeat in the HTT gene. HTT's exact function is still not fully understood, but previous studies have identified several interacting proteins that shed light on its function and structure. Among these proteins, HAP1 and HIP1 have been extensively studied, and recent research has found differences in their distribution in different animal brains. Understanding these species-specific variations in HTT-interacting proteins could provide crucial insights into HD development.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2023)
Review
Neurosciences
Kirby M. Donnelly, Cevannah M. Coleman, Madison L. Fuller, Victoria L. Reed, Dayna Smerina, David S. Tomlinson, Margaret M. Panning Pearce
Summary: This article reviews the evidence for prion-like mechanisms in Huntington's disease, emphasizing the importance of mutant HTT protein in disease propagation, and discusses the potential benefits of targeting such mechanisms in search of therapeutic approaches.
FRONTIERS IN NEUROSCIENCE
(2022)
Review
Biochemistry & Molecular Biology
Silvia Marti-Martinez, Luis M. Valor
Summary: Huntington's disease is a neurodegenerative disorder caused by abnormal expansion of CAG repeats in the Huntingtin gene. It not only affects the central nervous system but also has a peripheral component. Precise diagnosis and evaluation of therapeutic response require the use of biomarkers with prognostic value.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Article
Neurosciences
Neha Sawant, Hallie Morton, Sudhir Kshirsagar, Arubala P. Reddy, P. Hemachandra Reddy
Summary: Huntington's disease is a fatal genetic disease involving multiple cellular changes, with therapeutic strategies focusing on reducing abnormal protein interactions and enhancing synaptic mitophagy.
MOLECULAR NEUROBIOLOGY
(2021)
Review
Neurosciences
Subrata Pradhan, Rui Gao, Keegan Bush, Nan Zhang, Yogesh P. Wairkar, Partha S. Sarkar
Summary: Emerging evidence suggests a strong correlation between DNA repair deficiency, genome instability, and neurological diseases such as Huntington's disease. Research has shown that defective DNA repair plays a significant role in the progression of neurodegenerative diseases, and proteins linked to these diseases are involved in cellular pathways. This article focuses on the mechanisms by which the huntingtin protein helps DNA repair during transcription and how its mutations impede this process in Huntington's disease.
FRONTIERS IN CELLULAR NEUROSCIENCE
(2022)
Article
Clinical Neurology
Melanie Alpaugh, Francesca Cicchetti
Summary: Research suggests that Huntington's disease shares similarities with prion disorders and may also have sporadic forms, indicating potential common mechanisms governing these neurodegenerative disorders.
JOURNAL OF NEUROLOGY
(2021)
Review
Biochemistry & Molecular Biology
Aleksandra S. Churkina (Taran), Anton S. Shakhov, Anatoly A. Kotlobay, Irina B. Alieva
Summary: Neurodegenerative diseases cannot be cured, but they share similar cellular pathologies. This offers hope for finding common intracellular targets and universal treatment methods.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2022)
Review
Biochemistry & Molecular Biology
Karen A. Sap, Karlijne W. Geijtenbeek, Sabine Schipper-Krom, Arzu Tugce Guler, Eric A. Reits
Summary: Huntington's disease (HD) is a neurodegenerative disorder caused by a CAG repeat expansion in the HTT gene, resulting in the accumulation and toxicity of mutant HTT (mHTT) protein. The ubiquitin-proteasome system (UPS) is involved in clearing mHTT proteins, but the polyQ expansion in mHTT affects its interaction with ubiquitinating enzymes and targeting for degradation. Various ubiquitin-modifying enzymes have been identified that can improve mHTT turnover and overall homeostasis, providing potential targets for therapeutic intervention in HD.
FRONTIERS IN MOLECULAR BIOSCIENCES
(2023)
Review
Biochemistry & Molecular Biology
Hanadi Ananbeh, Petr Vodicka, Helena Kupcova Skalnikova
Summary: Exosomes may play a role in the spreading of toxic proteins in neurodegenerative diseases and carry molecules with neuroprotective effects. Due to their ability to cross the blood-brain barrier, exosomes hold potential as a source of biomarkers and carriers for HD diagnosis and therapy.
INTERNATIONAL JOURNAL OF MOLECULAR SCIENCES
(2021)
Article
Biochemistry & Molecular Biology
Ligia Fao, Ana Cristina Rego
Summary: The molecular processes underlying Huntington's disease (HD) pathogenesis remain unclear, with a key role identified for mitochondrial dysfunction and redox changes. Therapeutic strategies targeting these mechanisms may prevent selective neurodegeneration in HD.
ANTIOXIDANTS & REDOX SIGNALING
(2021)
Article
Clinical Neurology
Hongshuai Liu, Chuangchuang Zhang, Jiadi Xu, Jing Jin, Liam Cheng, Xinyuan Miao, Qian Wu, Zhiliang Wei, Peiying Liu, Hanzhang Lu, Peter C. M. van Zijl, Christopher A. Ross, Jun Hua, Wenzhen Duan
Summary: Huntington's disease is a fatal neurodegenerative disorder caused by a mutation in the HTT gene, and research using a mouse model has shown that changes in CBVa can be detected in the premanifest stage of the disease, with non-allele-specific HTT silencing showing promising therapeutic effects.
Article
Neurosciences
Ya Zhou, Thomas R. Peskett, Christian Landles, John B. Warner, Kirupa Sathasivam, Edward J. Smith, Shu Chen, Ronald Wetzel, Hilal A. Lashuel, Gillian P. Bates, Helen R. Saibil
Summary: Huntington's disease is a late onset neurodegenerative disorder, and mutant HTT proteins are recruited to specific endolysosomal organelles in presymptomatic stages, mainly multivesicular bodies and amphisomes. As the disease progresses, these organelles transition into autolysosomes or residual bodies.
ACTA NEUROPATHOLOGICA COMMUNICATIONS
(2021)