Journal: Journal of Biomedical Science

Article Title: Modeling CLN3 Batten disease in astrocytes reveals alterations in mitochondria homeostasis, fatty acid metabolism and oxidative stress response

doi: 10.1186/s12929-026-01253-y

Figure Lengend Snippet: Generation and characterization of iPSC-derived astrocytes. A Schematic overview of astrocyte differentiation from patient-derived iPSCs. Key compounds used to drive differentiation toward a mature astrocyte phenotype are indicated: LIF (leukemia inhibitory factor), CHIR99021, SB431542, CoE, FGF2 (fibroblast growth factor 2), EGF (epidermal growth factor), and CNTF (ciliary neurotrophic factor). B qPCR analysis of astrocyte-specific markers (ALDHL1, GLAST, S100b, GFAP, Vimentin) in iPSC-derived cells. Expression of MAP2 (neuron marker) and OLIG2 (oligodendrocyte marker) was assessed to evaluate cell population purity. C Phase-contrast images and immunocytochemical validation of astrocyte marker expression. Vimentin and GFAP (green), S100β (red), and DAPI-stained nuclei (blue) are shown. D Quantification of CLN3 protein levels in control and CLN3 patient-derived iPSC and astrocytes via targeted mass spectrometry

Article Snippet: Healthy control fibroblast lines (two cell lines) were obtained from ATCC (cat. number PCS-201—012) and the Coriell Institute (cat. number AG05836).

Techniques: Derivative Assay, Expressing, Marker, Biomarker Discovery, Staining, Control, Mass Spectrometry

Journal: Photoacoustics

Article Title: Generative adversarial networks-enhanced quasi-square wave modulated photoacoustic spectroscopy: A highly sensitive NH 3 detection method under strong background noise

doi: 10.1016/j.pacs.2026.100799

Figure Lengend Snippet: (a) Schematic of the experimental system; (b) Absorption line of NH 3 and the interfering gas; (c) Structure and sound pressure distribution of the DPAC; (d) Sound pressure distribution of the DPAC on the logarithmic scale; (e) Frequency response of the DPAC. DFBLD: distributed-feedback laser diode; DP: differential preamplifier; DPAC: differential photoacoustic cell; FC: flow controller; Mic: microphone; PC: pressure controller; RC: resonant cavity; BC: buffer cavity.

Article Snippet: A flow controller (FC) (LK2, Alicat Scientific, USA) and a pressure controller (PC) (YK9, Alicat Scientific, USA) were employed to maintain a constant flow rate and pressure, respectively.

Techniques:

Journal: Antioxidants

Article Title: Bioenergetic Signatures of DLD Deficiency: Dissecting PDHc- and α-KGDHc-Linked Defects

doi: 10.3390/antiox15010019

Figure Lengend Snippet: Mitochondrial respiration is impaired in fibroblasts derived from patients with DLD deficiency. Mitochondrial oxygen consumption was assessed in controls (Ctrl1 and Ctrl2) and patient (Pt1–Pt6) fibroblasts using high-resolution respirometry (Oroboros O2k). ( A ) Routine respiration; ( B ) maximal respiration calculated as the difference between FCCP-stimulated and α-chaconine–permeabilized rates; ( C ) complex I-linked respiration (NADH-linked respiration, N-pathway), calculated as the difference between ADP and α-chaconine; ( D ) complex II-linked respiration (NS-pathway) calculated as the difference between respiration after rotenone and α-chaconine addition; ( E ) effect of complex I inhibition, calculated as the difference between FCCP-stimulated and rotenone-inhibited respiration; and ( F ) complex I/complex II respiration ratio (complex I-linked activity divided by complex II-linked activity). Each open circle represents an independent experimental run (N = 4–8 repeats per sample). All data were normalized to cell number. Statistical analysis was performed using the Mann–Whitney U test. * p < 0.05 vs. Ctrl1; numerical p values (0.05 < p < 0.1) are indicated on the plots. Abbreviations: Ctrl, control; Pt, patient.

Article Snippet: Dermal fibroblast primary cell lines from six genetically confirmed patients with DLD deficiency were obtained from the Pediatric Metabolic Disease Unit, Sheba Medical Center (IRB# SMC-21-8644, Figure 1, Table 1, and ), as well as two control cell lines: a control human dermal fibroblast cell line was purchased from ATCC (PCS-201-012; Ctrl 1, Manassas, VA, USA), and a primary cell line from a 39-year-old healthy male (Ctrl 2).

Techniques: Derivative Assay, Inhibition, Activity Assay, MANN-WHITNEY, Control