ArticleActa neuropathologica communications2015
Early detection of structural abnormalities and cytoplasmic accumulation of TDP-43 in tissue-engineered skins derived from ALS patients.
Article in Acta neuropathologica communications, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 30 papers.
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Who cites it
30 citing papers in PubMed, 56 citations in OpenAlex.
- Proteomic Analysis of Corpora Amylacea Extracted From Post-mortem Brain of MAiD-end-of-life Sporadic ALS Patients.Brain and behavior · 2026Article
- Skin pathology in ALS: Diagnostic implications and biomarker potential.Biomolecules & biomedicine · 2025Review
- Self-Produced Brain-Like ECM From 3D-Cultured Dermal Fibroblasts Enhances Neuronal Growth and Survival.Biotechnology journal · 2025Article
- Initiating Separate Cultures of Keratinocytes and Melanocytes from a Single Biopsy.Methods in molecular biology (Clifton, N.J.) · 2025Article
- TDP-43-proteinopathy at the crossroads of tauopathy: on copathology and current and prospective biomarkers.Frontiers in cellular neuroscience · 2025Review
- Microvascular abnormalities in skin capillaries of individuals with amyotrophic lateral sclerosis.Scientific reports · 2024Article
- Bioengineering Human Upper Respiratory Mucosa: A Systematic Review of the State of the Art of Cell Culture Techniques.Bioengineering (Basel, Switzerland) · 2024Review
- In vivo diagnosis of TDP-43 proteinopathies: in search of biomarkers of clinical use.Translational neurodegeneration · 2024Review
- Medication use and risk of amyotrophic lateral sclerosis: using machine learning for an exposome-wide screen of a large clinical database.Amyotrophic lateral sclerosis & frontotemporal degeneration · 2024Article
- Spherical rotary cell seeding system for production of small-caliber tissue-engineered blood vessels with complex geometry.Scientific reports · 2023Article
- In Vitro Characterization of Motor Neurons and Purkinje Cells Differentiated from Induced Pluripotent Stem Cells Generated from Patients with Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay.Stem cells international · 2023Article
- Tissue Engineering for Gastrointestinal and Genitourinary Tracts.International journal of molecular sciences · 2022Review
- Tridimensional cell culture of dermal fibroblasts promotes exosome-mediated secretion of extracellular matrix proteins.Scientific reports · 2022Article
- Article
- Genitourinary Tissue Engineering: Reconstruction and Research Models.Bioengineering (Basel, Switzerland) · 2021Review
- Innovative Human Three-Dimensional Tissue-Engineered Models as an Alternative to Animal Testing.Bioengineering (Basel, Switzerland) · 2020Review
- Characterization of the phenotype with cognitive impairment and protein mislocalization in SCA34.Neurology. Genetics · 2020Article
- Human Organ-Specific 3D Cancer Models Produced by the Stromal Self-Assembly Method of Tissue Engineering for the Study of Solid Tumors.BioMed research international · 2020Review
- A Systematic Review of Suggested Molecular Strata, Biomarkers and Their Tissue Sources in ALS.Frontiers in neurology · 2019Review
- Modeling human bladder cancer.World journal of urology · 2018Review
Corrections and comments
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Authors and funding
13 authors at 4 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Amyotrophic lateral sclerosis (ALS) is an adult-onset disease characterized by the selective degeneration of motor neurons in the brain and spinal cord progressively leading to paralysis and death. Current diagnosis of ALS is based on clinical assessment of related symptoms. The clinical manifestations observed in ALS appear relatively late in the disease course after degeneration of a significant number of motor neurons. As a result, the identification and development of disease-modifying therapies is difficult. Therefore, novel strategies for early diagnosis of neurodegeneration, to monitor disease progression and to assess response to existing and future treatments are urgently needed. Factually, many neurological disorders, including ALS, are accompanied by skin changes that often precede the onset of neurological symptoms. Aiming to generate an innovative human-based model to facilitate the identification of predictive biomarkers associated with the disease, we developed a unique ALS tissue-engineered skin model (ALS-TES) derived from patient's own cells. The ALS-TES presents a number of striking features including altered epidermal differentiation, abnormal dermo-epidermal junction, delamination, keratinocyte infiltration, collagen disorganization and cytoplasmic TDP-43 inclusions. Remarkably, these abnormal skin defects, uniquely seen in the ALS-derived skins, were detected in pre-symtomatic C9orf72-linked ALS patients carrying the GGGGCC DNA repeat expansion. Consequently, our ALS skin model could represent a renewable source of human tissue, quickly and easily accessible to better understand the physiophatological mechanisms underlying this disease, to facilitate the identification of disease-specific biomarkers, and to develop innovative tools for early diagnosis and disease monitoring.
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