Jervell and Lange‐Nielsen Syndrome Related Clinical Genetics and Experimental Models
ABSTRACT Jervell and Lange‐Nielsen syndrome (JLNS) is defined by electrocardiographic QT prolongation and sensorineural hearing loss, caused by homozygous or compound heterozygous variants in KCNQ1 and/or KCNE1. KCNQ1 encodes the alpha subunit Kv7.1 of the ion channels accountable for slow delayed rectifier potassium currents (IKs), whereas KCNE1 ...
Yafei Zhou +3 more
wiley +1 more source
Biochemical and Genetic Studies of UBR3, a Ubiquitin Ligase with a Function in Olfactory and Other Sensory Systems [PDF]
Our previous work identified E3 ubiquitin ligases, termed UBR1-UBR7, that contain the ~70-residue UBR box, a motif important for the targeting of N-end rule substrates.
Hellweg, Rainer +6 more
core +1 more source
Static length changes of cochlear outer hair cells can tune low-frequency hearing.
The cochlea not only transduces sound-induced vibration into neural spikes, it also amplifies weak sound to boost its detection. Actuators of this active process are sensory outer hair cells in the organ of Corti, whereas the inner hair cells transduce ...
Nikola Ciganović +5 more
doaj +1 more source
Practical Methodology for Developing Visions for Carbon‐Neutral Cities and Communities
ABSTRACT Cities and communities play important roles in sustainable development and climate change mitigation. This study presents a novel framework for developing carbon‐neutral visions for cities and communities. We established five “principles,” investigated options encompassing four parameters (management, goal setting, actions, and quantification),
Kei Gomi +4 more
wiley +1 more source
3D Ultrastructure of the Cochlear Outer Hair Cell Lateral Wall Revealed By Electron Tomography. [PDF]
Outer Hair Cells (OHCs) in the mammalian cochlea display a unique type of voltage-induced mechanical movement termed electromotility, which amplifies auditory signals and contributes to the sensitivity and frequency selectivity of mammalian hearing ...
Auer, Manfred +6 more
core +3 more sources
Six1 is essential for differentiation and patterning of the mammalian auditory sensory epithelium.
The organ of Corti in the cochlea is a two-cell layered epithelium: one cell layer of mechanosensory hair cells that align into one row of inner and three rows of outer hair cells interdigitated with one cell layer of underlying supporting cells along ...
Ting Zhang +3 more
doaj +1 more source
Comorbidity Patterns Between Hearing Loss and Symptomatic Dizziness in Middle‐Aged and Older Adults
ABSTRACT Objective To explore the comorbidity patterns between hearing loss and symptomatic dizziness (HL‐SD) in middle‐aged and older adults. Methods This cross‐sectional study used data from the 1999 to 2004 National Health and Nutrition Examination Survey (NHANES). After excluding incomplete data, 2961 participants aged 40–69 were analyzed. Logistic
Chun‐Yan Liu +6 more
wiley +1 more source
Epac1 Alleviates Senescence in Auditory Hair Cells via the Ferroptosis
ABSTRACT Objectives The aim of this study was to investigate the changes in Epac1 and ferroptosis‐related proteins in aged mice and the House Ear Institute‐Organ of Corti 1 (HEI‐OC1) cells, with the goal of elucidating their potential role in age‐related hearing loss. Methods The expression of Epac1 in the cochlea of C57BL/6J mice was examined by using
Wen‐Jun An +7 more
wiley +1 more source
Assembly of the Auditory Circuitry by a Hox Genetic Network in the Mouse Brainstem [PDF]
Rhombomeres (r) contribute to brainstem auditory nuclei during development. Hox genes are determinants of rhombomere-derived fate and neuronal connectivity.
Andolfi, G. +9 more
core +5 more sources
Background Inhibitors of 3-hydroxy-3-methylglutaryl-coenzyme A reductase, known as statins, are commonly used as cholesterol-lowering drugs. During the past decade, evidence has emerged that statins also have neuroprotective effects.
Brand Yves +9 more
doaj +1 more source

