Phenotypic Heterogeneity in Patients with Mutations in the Mitochondrial Complex I Assembly Gene NDUFAF5.

Background: Rare mutations in NADH:ubiquinone oxidoreductase complex assembly factor 5 (NDUFAF5) are linked to Leigh syndrome.

Objective: We aimed to describe clinical characteristics and functional findings in a patient cohort with NDUFAF5 mutations.

Methods: Patients with biallelic NDUFAF5 mutations were recruited from multi-centers in Taiwan.

Cell Types Promoting Goosebumps Form a Niche to Regulate Hair Follicle Stem Cells.

Piloerection (goosebumps) requires concerted actions of the hair follicle, the arrector pili muscle (APM), and the sympathetic nerve, providing a model to study interactions across epithelium, mesenchyme, and nerves. Here, we show that APMs and sympathetic nerves form a dual-component niche to modulate hair follicle stem cell (HFSC) activity. Sympathetic nerves form synapse-like structures with HFSCs and regulate HFSCs through norepinephrine, whereas APMs maintain sympathetic innervation to HFSCs.

Proteomic Analysis Reveals Anti-Fibrotic Effects of Blue Light Photobiomodulation on Fibroblasts.

Background And Objectives: This study was aimed at determining the effects of blue light photobiomodulation on primary adult mouse dermal fibroblasts (AMDFs) and the associated signaling pathways.

Study Design/materials And Methods: Cultured AMDFs from adult C57BL/6 mice were irradiated by blue light from a light-emitting diode (wavelength = 463 ± 50 nm; irradiance = 5 mW/cm ; energy density = 4-8 J/cm ). The cells were analyzed using mass spectrometry for proteomics/phosphoproteomics, AlamarBlue assay for mitochondrial activity, time-lapse video for cell migration, quantitative polymerase chain reaction for gene expression, and immunofluorescence for protein expression.

External light activates hair follicle stem cells through eyes via an ipRGC-SCN-sympathetic neural pathway.

Changes in external light patterns can alter cell activities in peripheral tissues through slow entrainment of the central clock in suprachiasmatic nucleus (SCN). It remains unclear whether cells in otherwise photo-insensitive tissues can achieve rapid responses to changes in external light. Here we show that light stimulation of animals' eyes results in rapid activation of hair follicle stem cells with prominent hair regeneration.

Inducing hair follicle neogenesis with secreted proteins enriched in embryonic skin.

Organ development is a sophisticated process of self-organization. However, despite growing understanding of the developmental mechanisms, little is known about how to reactivate them postnatally for regeneration. We found that treatment of adult non-hair fibroblasts with cell-free extract from embryonic skin conferred upon them the competency to regenerate hair follicles.

Preclinical evaluation of melanocyte transplantation by chitosan-based melanocyte spheroid patch to skin prepared by controlled sunburn blistering.

Transplantation of autologous cultured melanocytes as cell suspension has been used for the treatment of vitiligo. The recipient site is often prepared by laser-mediated dermabrasion. Such procedures encounter disadvantages including prolonged transplantation duration, unsecured cell adherence to lesional skin and potential scarring.

Activation of mTORC1 Signaling is Required for Timely Hair Follicle Regeneration from Radiation Injury.

Transit amplifying cells (TACs) are highly proliferative in nature and tend to be sensitive to ionizing radiation. Due to the abundance of TACs that support the elongation of hair shafts, growing hair follicles are highly sensitive to radiation injury. How hair follicles repair themselves after radiation injury is unclear.

Programmable Laser-Assisted Surface Microfabrication on a Poly(Vinyl Alcohol)-Coated Glass Chip with Self-Changing Cell Adhesivity for Heterotypic Cell Patterning.

Organs are composed of heterotypic cells with patterned architecture that enables intercellular interaction to perform specific functions. In tissue engineering, the ability to pattern heterotypic cells into desired arrangement will allow us to model complex tissues in vitro and to create tissue equivalents for regeneration. This study was aimed at developing a method for fast heterotypic cell patterning with controllable topological manipulation on a glass chip.

A Two-Stepped Culture Method for Efficient Production of Trichogenic Keratinocytes.

Successful hair follicle (HF) neogenesis in adult life depends on the existence of both capable dermal cells and competent epidermal keratinocytes that recapitulate embryonic organogenesis through epithelial-mesenchymal interaction. In tissue engineering, the maintenance of trichogenic potential of adult epidermal cells, while expanding them remains a challenging issue. We found that although HF outer root sheath keratinocytes could be expanded for more than 100 passages as clonogenic cells without losing the proliferative potential with a 3T3J2 fibroblast feeder layer, these keratinocytes were unable to form new HFs when combined with inductive HF dermal papilla (DP) cells.

Enhancing hair follicle regeneration by nonablative fractional laser: Assessment of irradiation parameters and tissue response.

Background And Objective: Identification of methods to enhance anagen entry can be helpful for alopecia. Recently, nonablative laser has been proposed as a potential treatment for alopecia. However, how the laser parameters affect stem cell activity, hair cycles and the associated side effects have not been well characterized.

Visible red light enhances physiological anagen entry in vivo and has direct and indirect stimulative effects in vitro.

Background And Objectives: Hair follicles are located at the interface of the external and internal environments and their cycling has been shown to be regulated by intra- and extra-follicular factors. The aim of this study is to examine whether or how hair follicles respond to visible light.

Study Design/materials And Methods: We examined the effect of 3 mW red (630 nm, 1 J/cm(2)), 2 mW green (522 nm, 1 J/cm(2)), and 2 mW blue light (463 nm, 1 J/cm(2)) on telogen in mice for 3 weeks.

Toona sinensis Roem (Meliaceae) leaf extract alleviates liver fibrosis via reducing TGFbeta1 and collagen.

Toona sinensis Roem (TS) leaf tea as a health food for the improvement of blood sugar and hypertension has been demonstrated. Thioacetamide (TAA), a hepatotoxin, causes the progression of liver fibrosis. In this study, we tested the effects of TS leaf on TAA-induced liver injury.

Down-regulation of TGFbeta1 and leptin ameliorates thioacetamide-induced liver injury in lipopolysaccharide-primed rats.

Pretreatment with a low dose of bacterial endotoxin (lipopolysaccharide, LPS) caused the reduction of cytochrome P450 (CYP) enzymes and inflammatory factors which are capable of protecting the liver from a lethal LPS challenge. However, the effects of LPS pretreatment on the expression of transforming growth factor beta1 (TGFbeta1) and leptin in thioacetamide (TAA)-induced liver fibrosis remain unknown. In this study, Sprague-Dawley rats were pretreated intraperitoneally with LPS (5 mg/kg body weight) for 24 h, and subsequently treated with TAA (200 mg/kg body weight/ 3 days) for 1 month to examine the effects of LPS on TAA-injured rats.

Co-administration of cyclosporine an alleviates thioacetamide-induced liver injury.

Aim: To investigate the effects of cyclosporine A (CsA) on thioacetamide (TAA)-induced liver injury.

Methods: CsA was co-administrated (7.5 microg/kg body weight per day, i.