Journal: Journal of Neuroinflammation

Article Title: Early control of cochlear viral load limits cochlear inflammation and prevents virus-induced sensorineural hearing loss

doi: 10.1186/s12974-025-03416-4

Figure Lengend Snippet: Treatment with anti-MCMV mAbs prevents decreased expression of deafness-related genes in the cochlea during early auditory development. (A-F) Validation of decreased expression of select deafness related genes identified by RNA-seq. Cochleae collected on PNd4 and expression of Cx26, Cx43, KCNE1, ZBTB20, COCH, and OTOS determined by qRT-PCR as described in Fig. . Significant decrease of DRG expression in cochleae from MCMV-infected, untreated (MCMV) and isotype control antibody treated cochleae (MCMV + ISO) as compared to cochleae from infected mAb treated (MCMV + mAb) and uninfected control mice (MOCK) for each of the selected genes with exception of ZBTB20. Each data point represents individual cochlea with median of group indicated by horizontal bar. All groups compared to MOCK and statistical significance determined by a Kruskal-Wallis test with Dunn’s multiple comparisons post-test ( * P < 0.05, ** P < 0.01). For each group, n = 3–5 mice (6–10 cochleae)

Article Snippet: TaqMan Gene Expression Master Mix (ThermoFisher) was used for qPCR of specific gene targets: HPRT (Mm00446968_m1), IFIT1 (Mm00515153_m1), TNF (Mm00443258_m1), IFNg (Mm01168134_m1), IL1b (Mm00434228_m1), GJB2 (Mm00433643_s1), GJA1 (Mm00439105_m1), KCNE1 (Mm01215533_m1), ZBTB20 (Mm00457764_m1), COCH (Mm00483360_m1), and OTOS (Mm01292235_g1).

Techniques: Expressing, Biomarker Discovery, RNA Sequencing, Quantitative RT-PCR, Infection, Control

Journal: Cell and Tissue Research

Article Title: Connexins and gap junctions in the inner ear – it’s not just about K + recycling

doi: 10.1007/s00441-014-2029-z

Figure Lengend Snippet: Expression patterns of Cx26 and Cx30 in epithelial and connective tissues of the cochlea. a A section of the cochlear apical turn of a P30 mouse showing the key sensory and non-sensory tissues involved in auditory sensory transduction. b Higher magnification of the organ of Corti showing the inner hair cells and outer hair cells separated by the tunnel of Corti, formed by the inner pillar cells and outer pillar cells. Adjacent to the outer hair cells are the Deiters’ cells, Hensen’s cells and Claudius’ cells. Immunofluorescent labelling of the organ of Corti ( c ) for Cx26 ( green ) and Cx30 ( red ) reveals a differential pattern of the connexins. Nuclei are stained using DAPI. Within the Deiters’ cell region, there are mostly Cx30-labelled gap junction plaques, whereas double-labelled plaques are evident elsewhere. In the connective tissue region of the cochlear lateral wall ( d ), most gap junction plaques are double-labelled for Cx26 and Cx30. anf auditory nerve fibres, bc basal cell, Cc Claudius’ cell, Dc Deiters’ cell, ec endothelial cell, fc fibrocyte, Hc Hensen’s cell, ic intermediate cell, ihc inner hair cell, ipc inner pillar cell, mc marginal cell, oC organ of Corti, ohc outer hair cell, opc outer pillar cell, Rm Reissner’s membrane, sg spiral ganglion, sl spiral ligament, sv stria vascularis, sm scala media, tm tectorial membrane. Scale bars 20 μm

Article Snippet: Further complications may also arise following the identification of pathogenic mutations outside the coding region of GJB2 (Matos et al. ).

Techniques: Expressing, Transduction, Staining, Membrane

Journal: Cell and Tissue Research

Article Title: Connexins and gap junctions in the inner ear – it’s not just about K + recycling

doi: 10.1007/s00441-014-2029-z

Figure Lengend Snippet: Cx26 and Cx30 play specific roles in development, membrane function and epithelial repair of the cochlea. a , b Light microscopy images of cochlear tissues. a In the basal turn of a 2-week old R75W- Cx26 mouse, the essential sensory and non-sensory tissues are present, but on closer inspection the organ of Corti appears compact ( a’ ). Deiters’ cells are short and intercellular spaces such as the tunnel of Corti and spaces of Nuel are absent. b In comparison, in a juvenile wild-type mouse, the organ of Corti has elongated Deiters’ cells and pillar cells, and a fully patent tunnel of Corti (*). c , d Freeze-fracture images of Deiters’ cell membranes in mice. Freeze-fracture reveals gap junction plaques as clusters of particles in the membrane. Each particle represents an individual channel (connexon). c In Cx30- null animals, plaques of the gap junctions between adjacent Deiters’ cells consist of only a few channels, and they are dispersed. d In wild-type animals, the plaques are extremely large (consisting of thousands of channels) and occupy a significant proportion of the membrane area. e , f Transmission electron micrographs of cochlear tissues. e Following loss of outer hair cells from a Cx30 -null mouse, the Deiters’ cells remain columnar in shape, the tunnel of Corti is open (*), and the reticular lamina is displaced towards the pillar cells ( arrow ). f In a wild-type mouse treated systemically with kanamycin and bumetanide to cause loss of inner and outer hair cells, the Deiters’ cells have expanded to fill the gaps left behind by the hair cells. Cells with characteristics of Deiters’ cells have migrated between the outer pillar cells so that the tunnel of Corti appears filled (*). Dc Deiters’ cell, oC organ of Corti, Rm Reissner’s membrane, sg spiral ganglion, sv stria vascularis, Tc tectal cell. Scale bars ( a , b , e , f ) 10 μm, ( c , d ) 0.1 μm

Article Snippet: Further complications may also arise following the identification of pathogenic mutations outside the coding region of GJB2 (Matos et al. ).

Techniques: Membrane, Light Microscopy, Comparison, Transmission Assay

Journal: Scientific Reports

Article Title: The genetic basis and the diagnostic yield of genetic testing related to nonsyndromic hearing loss in Qatar

doi: 10.1038/s41598-024-52784-z

Figure Lengend Snippet: The Utilization frequencies of the different genetic tests and their associated diagnostic yields.

Article Snippet: At Hamad Medical Corporation (HMC), the main health care hospital in Qatar, clinical approach to genetic testing for HL aligns with the ACMG recommendations previously mentioned: GJB2 gene sequencing is offered as first-tier genetic testing with or without chromosomal microarray, and HL gene panel or WES are offered as second-tier genetic workup.

Techniques: Diagnostic Assay, Sequencing, Microarray, Variant Assay

Journal: Scientific Reports

Article Title: The genetic basis and the diagnostic yield of genetic testing related to nonsyndromic hearing loss in Qatar

doi: 10.1038/s41598-024-52784-z

Figure Lengend Snippet: Variants reported in the solved cases (n = 20).

Article Snippet: At Hamad Medical Corporation (HMC), the main health care hospital in Qatar, clinical approach to genetic testing for HL aligns with the ACMG recommendations previously mentioned: GJB2 gene sequencing is offered as first-tier genetic testing with or without chromosomal microarray, and HL gene panel or WES are offered as second-tier genetic workup.

Techniques: Variant Assay, Sequencing