Huntington’s disease (HD) is a paradigm of a genetic neurodegenerative disorder characterized by the expansion of CAG repeats in theHTTgene. This extensive review investigates the molecular complexities of HD by highlighting the pathogenic mechanisms initiated by the mutant huntingtin protein. Adverse o...
Huntington’s disease HI: Hypoxia-ischemia HIE: Hypoxic-ischemic encephalopathy 4-HNE: 4-hydroxynonenal HO-1: Heme oxygenase 1 HP: Hippocampus HT: Hypothalamus ICH: Intracerebral hemorrhage iNOS: Inducible nitric oxide synthase IQ: Intelligence quotient KEAP 1: Kelch-like ECH-associated...
Proteinopathies is a set of neurodegenerative diseases characterized by the accumulation of protein aggregates in the central nervous system and include Alzheimer’s, Parkinson’s and Huntington’s disease; and amyotrophic lateral sclerosis. • The central players of aggrephagy are the selective autoph...
A videotaped neurological examination was performed in 15 of the 20 patients, using three standardized scales—the Clinical Rating Scale for Tremors (CRST) (Fahn et al. 1998), the motor section of the Unified Parkinson’s Disease Rating Scale (UPDRS) (Fahn et al. 1987), and the ...
Iron is an essential element for the development and functionality of the brain, and anomalies in its distribution and concentration in brain tissue have been found to be associated with the most frequent neurodegenerative diseases. When magnetic resonan
neurodegenerative diseases. This review presents insights into the roles played by nuclear transport defects in neurodegenerative disease, focusing primarily on NE proteins and NPCs. The subcellular mislocalization of proteins might be a very desirable means of therapeutic intervention in neurodegenerative ...
Neurodegenerative diseases, such as Parkinson’s disease (PD), Alzheimer’s disease (AD), Huntington’s disease (HD), and amyotrophic lateral sclerosis (ALS), are characterized by the progressive loss of neurons and/or neuronal functions. These syndromes are developed by the presence of aggregate...
The discovery of insulin 100 years ago and its application to the treatment of human disease in the years since have marked a major turning point in the history of medicine. The availability of purified insulin allowed for the establishment of its physiological role in the regulation of blood gl...
Ohnishi S, Takano K (2004) Amyloid fibrils from the viewpoint of protein folding. Cell Mol Life Sci 61:511–524. doi:10.1007/s00018-003-3264-8 Article PubMed CAS Google Scholar Chen S, Ferrone FA, Wetzel R (2002) Huntington’s disease age-of-onset linked to polyglutamine aggregation...
In recent years, neurological disorders have globally become a leading cause of disability and death. Neurological disorders are very common in both high-