Abnormal apocrine secretory cell mitochondria in a Huntington disease patient

Sidiropoulos, Christos; LeWitt, Peter A.; Hashimoto, Ken

doi:10.1016/j.jns.2012.08.034

Cited by 2 publications

(1 citation statement)

References 23 publications

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“…For example, patients with sporadic amyotrophic lateral sclerosis (SALS) present mitochondrial dysfunction not only in the central nervous system, but also in skin biopsies [211,212]. Additionally, in the mitochondrial neurodegenerative Huntington's disease (HD) (OMIM #143100), skin biopsies can show hyperkeratosis, epidermal atrophy, subepidermal fibrosis, increased acid mucopolysaccharides [213] and disrupted mitochondrial cristae [214], even though obvious macroscopic cutaneous changes are generally absent. Thus, dermatopathology may facilitate early diagnosis of selected neurodegenerative diseases.…”

Section: Mitochondrial Dermatology 18mentioning

confidence: 99%

Targeting mitochondria in dermatological therapy: beyond oxidative damage and skin aging

Wikramanayake

Chéret

Sevilla

et al. 2022

Expert Opinion on Therapeutic Targets

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Introduction:The analysis of the role of the mitochondria in oxidative damage and skin aging is a significant aspect of dermatological research. Mitochondria generate most reactive oxygen species (ROS); however, excessive ROS are cytotoxic and DNA-damaging and promote (photo-)aging. ROS also possesses key physiological and regulatory functions and mitochondrial dysfunction is prominent in several skin diseases including skin cancers. Although many standard dermatotherapeutics modulate mitochondrial function, dermatological therapy rarely targets the mitochondria. Accordingly, there is a rationale for "mitochondrial dermatology"-based approaches to be applied to therapeutic research.Areas covered: This paper examines the functions of mitochondria in cutaneous physiology beyond energy (ATP) and ROS production. Keratinocyte differentiation and epidermal barrier maintenance, appendage morphogenesis and homeostasis, photoaging and skin cancer are considered. Based on related PubMed search results, the paper evaluates thyroid hormones, glucocorticoids, Vitamin D3 derivatives, retinoids, cannabinoid receptor agonists, PPARγ agonists, thyrotropin, and thyrotropinreleasing hormone as instructive lead compounds. Moreover, the mitochondrial protein MPZL3 as a promising new drug target for future "mitochondrial dermatology" is highlighted.Expert opinion: Future dermatological therapeutic research should have a mitochondrial medicine emphasis. Focusing on selected lead agents, protein targets, in silico drug design, and model diseases will fertilize a mito-centric approach.

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Section: Mitochondrial Dermatology 18mentioning

confidence: 99%

Targeting mitochondria in dermatological therapy: beyond oxidative damage and skin aging

Wikramanayake

Chéret

Sevilla

et al. 2022

Expert Opinion on Therapeutic Targets

View full text Add to dashboard Cite

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Huntington’s Disease: Pathogenic Mechanisms and Therapeutic Targets

Wright

Renoir

Gray

et al. 2017

Advances in Neurobiology

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Huntington's disease (HD) is a tandem repeat disorder involving neurodegeneration and a complex combination of symptoms. These include psychiatric symptoms, cognitive deficits culminating in dementia, and the movement disorder epitomised by motor signs such as chorea. HD is caused by a CAG repeat expansion encoding an extended tract of the amino acid glutamine in the huntingtin protein. This polyglutamine expansion appears to induce a 'change of function', possibly a 'gain of function', in the huntingtin protein, which leads to various molecular and cellular cascades of pathogenesis. In the current review, we will briefly describe these broader aspects of HD pathogenesis, but will then focus on specific aspects where there are substantial bodies of experimental evidence, including oxidative stress, mitochondrial dysfunction, glutamatergic dysfunction and neuroinflammation. Furthermore, we will review recent preclinical therapeutic approaches targeting some of these pathogenic pathways, their clinical implications and future directions.

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Abnormal apocrine secretory cell mitochondria in a Huntington disease patient

Cited by 2 publications

References 23 publications

Targeting mitochondria in dermatological therapy: beyond oxidative damage and skin aging

Targeting mitochondria in dermatological therapy: beyond oxidative damage and skin aging

Huntington’s Disease: Pathogenic Mechanisms and Therapeutic Targets

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