Medical, Surgical, and Genetic Treatment of Huntington Disease

Stahl, Christine M.; Feigin, Andrew

doi:10.1016/j.ncl.2020.01.010

Cited by 32 publications

(17 citation statements)

References 52 publications

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“…Till now, there is no treatment to cure HD, and the clinic treatments only could alleviate symptoms to improve the quality of life (Table 1 ). Chorea is one of motor symptoms in HD, and decreased neurotransmission of dopamine has shown the reduction of chorea [ 40 ], indicating decrease of dopamine signaling is a therapeutic target for HD patients. Tetrabenazine (TBZ) and Deutetrabenazine, both approved by the Food and Drug Administration (FDA), are inhibitors of the vesicular monoamine transporter type 2 (VMAT2), and functions through depletion of dopamine in the presynaptic terminals to improve chorea [ 40 , 41 ].…”

Section: Huntington’s Diseasementioning

confidence: 99%

“…Chorea is one of motor symptoms in HD, and decreased neurotransmission of dopamine has shown the reduction of chorea [ 40 ], indicating decrease of dopamine signaling is a therapeutic target for HD patients. Tetrabenazine (TBZ) and Deutetrabenazine, both approved by the Food and Drug Administration (FDA), are inhibitors of the vesicular monoamine transporter type 2 (VMAT2), and functions through depletion of dopamine in the presynaptic terminals to improve chorea [ 40 , 41 ]. In addition, dopamine receptor blockers, such as tiapride, haloperidol, fluphenazine, olanzapine and risperidone, are also used to treat HD to suppress chorea.…”

Section: Huntington’s Diseasementioning

confidence: 99%

“…In addition, dopamine receptor blockers, such as tiapride, haloperidol, fluphenazine, olanzapine and risperidone, are also used to treat HD to suppress chorea. Since dopamine receptor blockers belong to one type of antipsychotics, these drugs also alleviate comorbid psychiatric symptoms in HD [ 40 , 42 ]. Besides to the decrease of dopamine signaling, suppression of glutamate transmission is also a strategy to reduce chorea.…”

Section: Huntington’s Diseasementioning

confidence: 99%

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The regulatory roles of microRNAs toward pathogenesis and treatments in Huntington's disease

et al. 2021

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Huntington’s disease (HD) is one of neurodegenerative diseases, and is defined as a monogenetic disease due to the mutation of Huntingtin gene. This disease affects several cellular functions in neurons, and further influences motor and cognitive ability, leading to the suffering of devastating symptoms in HD patients. MicroRNA (miRNA) is a non-coding RNA, and is responsible for gene regulation at post-transcriptional levels in cells. Since one miRNA targets to several downstream genes, it may regulate different pathways simultaneously. As a result, it raises a potential therapy for different diseases using miRNAs, especially for inherited diseases. In this review, we will not only introduce the update information of HD and miRNA, but also discuss the development of potential miRNA-based therapy in HD. With the understanding toward the progression of miRNA studies in HD, we anticipate it may provide an insight to treat this devastating disease, even applying to other genetic diseases.

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Section: Huntington’s Diseasementioning

confidence: 99%

Section: Huntington’s Diseasementioning

confidence: 99%

Section: Huntington’s Diseasementioning

confidence: 99%

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The regulatory roles of microRNAs toward pathogenesis and treatments in Huntington's disease

et al. 2021

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“…It is estimated that the mean HD prevalence is 5 in 100,000 people ( Baig et al, 2016 ; Illarioshkin et al, 2018 ). HD is characterized by a neurobehavioral progressive triad with motor dysfunction, psychiatric disturbance, and cognitive decline ( Stahl and Feigin, 2020 ). The motor dysfunction is subdivided into two stages: In the early stage, there are abnormal involuntary movements, known as chorea, while in the late stage, the voluntary movements are impaired, causing bradykinesia, dystonia, and motor incoordination.…”

Section: Introductionmentioning

confidence: 99%

“…The cognitive alterations include impaired attention and visuospatial functions and slow planning processing speed. The cognitive decline progresses to dementia ( Stahl and Feigin, 2020 ), and death becomes imminent 15–20 years after disease onset ( Blumenstock and Dudanova, 2020 ). These dysfunctions can be attributed to multiple brain regions that exhibit neurodegeneration, including the cerebral cortex, thalamus, subthalamic nucleus, globus pallidus, substantia nigra, and hypothalamus.…”

Section: Introductionmentioning

confidence: 99%

Purinergic Signaling in the Pathophysiology and Treatment of Huntington’s Disease

Wiprich

Bonan

2021

Front. Neurosci.

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Huntington’s disease (HD) is a devastating, progressive, and fatal neurodegenerative disorder inherited in an autosomal dominant manner. This condition is characterized by motor dysfunction (chorea in the early stage, followed by bradykinesia, dystonia, and motor incoordination in the late stage), psychiatric disturbance, and cognitive decline. The neuropathological hallmark of HD is the pronounced neuronal loss in the striatum (caudate nucleus and putamen). The striatum is related to the movement control, flexibility, motivation, and learning and the purinergic signaling has an important role in the control of these events. Purinergic signaling involves the actions of purine nucleotides and nucleosides through the activation of P2 and P1 receptors, respectively. Extracellular nucleotide and nucleoside-metabolizing enzymes control the levels of these messengers, modulating the purinergic signaling. The striatum has a high expression of adenosine A2A receptors, which are involved in the neurodegeneration observed in HD. The P2X7 and P2Y2 receptors may also play a role in the pathophysiology of HD. Interestingly, nucleotide and nucleoside levels may be altered in HD animal models and humans with HD. This review presents several studies describing the relationship between purinergic signaling and HD, as well as the use of purinoceptors as pharmacological targets and biomarkers for this neurodegenerative disorder.

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Insights into Advances and Applications of Biomaterials for Nerve Tissue Injuries and Neurodegenerative Disorders

Pai,

Singh,

Singh

2024

Macromolecular Bioscience

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The incidence of nerve tissue injuries, such as peripheral nerve injury, spinal cord injury, traumatic brain injury, and various neurodegenerative diseases (NDs), is continuously increasing because of stress, physical and chemical trauma, and the aging population worldwide. Restoration of the damaged nervous system is challenging because of its structural and functional complexity and limited regenerative ability. Additionally, there is no cure available for NDs except for medications that provide symptomatic relief. Stem cells offer an alternative approach for promoting damage repair, but their efficacy is limited by a compromised survival rate and neurogenesis process. To address these challenges, neural tissue engineering has emerged as a promising strategy in which stem cells are seeded or encapsulated within a suitable biomaterial construct, increasing cell survival and neurogenesis. Numerous biomaterials are utilized to create different types of constructs for this purpose. Researchers are trying to develop ideal scaffolds that combine biomaterials, cells, and molecules that exactly mimic the biological and mechanical properties of the tissue to achieve functional recovery associated with neurological dysfunction. This review focuses on exploring the development and applications of different biomaterials for their potential use in the diagnosis, therapy, nerve tissue regeneration, and treatment of neurological disorders.

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Medical, Surgical, and Genetic Treatment of Huntington Disease

Cited by 32 publications

References 52 publications

The regulatory roles of microRNAs toward pathogenesis and treatments in Huntington's disease

The regulatory roles of microRNAs toward pathogenesis and treatments in Huntington's disease

Purinergic Signaling in the Pathophysiology and Treatment of Huntington’s Disease

Insights into Advances and Applications of Biomaterials for Nerve Tissue Injuries and Neurodegenerative Disorders

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