K(+) currents through ERG (ether-à-go-go related gene) channels were recorded in whole-cell voltage clamped NG108-15 neuroblastomaxglioma hybrid cells. The channels were fully activated by low holding potential (V(H)=-20 mV) and long depolarizing prepulses. Hyperpolarizing pulses elicited inward currents which deactivated after reaching a peak. Lowering [Ca(2+)](o) from 5 to 1. 5 or 0.5 mM decreased tau(-1), the rate constant of deactivation. The effect can be explained by a shift of the tau(-1)(V) curve to more negative potentials caused by an increase in surface charge density. Plotting tau(-1) against [Ca(2+)](o) for different potentials yielded straight lines; their slope was independent of potential at -140 to -120 mV and decreased at more positive potentials. The time to peak curve and the maximum of the steady-state inward current were also shifted to more negative potentials. In addition, peak ERG inward current increased. Raising [Ca(2+)](o) from 5 to 10 mM accelerated deactivation and decreased the peak current. 5 mM Ba(2+) affected tau(-1) similarly and inhibited peak current more strongly whereas 5 mM Mg(2+) was less potent. As found by Faravelli et al. (J. Physiol. 496 (1996) 13), bath solutions devoid of divalent cations (0 Ca(2+), 0 Mg(2+), 0.1 or 1.1 mM EGTA) abolished deactivation almost completely. The phenomenon was seen with bath containing either 40 or 6.5 mM K(+). Its occurrence was favored by raising the temperature to 34 degrees C. It suggests a particular requirement of channel closing for Ca(2+).
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http://dx.doi.org/10.1016/s0005-2736(00)00302-3 | DOI Listing |
J Neuroinflammation
January 2025
Department of Ophthalmology, Wilmer Eye Institute, Johns Hopkins University School of Medicine, Baltimore, MD, 21231, USA.
Background: The retinal degenerative diseases retinitis pigmentosa (RP) and atrophic age- related macular degeneration (AMD) are characterized by vision loss from photoreceptor (PR) degeneration. Unfortunately, current treatments for these diseases are limited at best. Genetic and other preclinical evidence suggest a relationship between retinal degeneration and inflammation.
View Article and Find Full Text PDFOphthalmic Genet
January 2025
Department of Ophthalmology, Northwestern University Feinberg School of Medicine, Chicago, Illinois, USA.
Introduction: Due to the recent advent of gene-targeted retinal therapies, the clinical value of high-yield genetic testing for inherited retinal dystrophies (IRDs) has increased considerably. However, diagnostic yield is limited by the reported patient populations in allele frequency databases. This study aimed to determine the effect of race and ethnicity on diagnostic yield in IRDs.
View Article and Find Full Text PDFSynth Syst Biotechnol
November 2024
School of Chemical Engineering and Technology, Hebei University of Technology, Tianjin, 300401, China.
Ergothioneine (ERG), a rare natural thio-histidine derivative with potent antioxidant properties and diverse biological functions, is widely utilized in food processing, cosmetics, pharmaceuticals, and nutritional supplements. Current bioproduction methods for ERG primarily depend on fermenting edible mushrooms. However, with the advancement in synthetic biology, an increasing number of genetically engineered microbial hosts have been developed for ERG production, including , , and .
View Article and Find Full Text PDFForensic Sci Int Genet
December 2024
Estonian Forensic Science Institute, Tallinn, Estonia.
The utilisation of massively parallel sequencing (MPS) in forensic DNA analysis is on the rise, driven by the expansion of targeted MPS panels in the market and the introduction of forensic investigative genetic genealogy. The MPS library preparation process, integral to both whole-genome sequencing (WGS) and targeted MPS panel data generation, is largely based on converting double-stranded DNA (dsDNA) into sequencing libraries. In the current study, we examined the effect of seven routinely used forensic DNA extraction methods on the strandedness (single-stranded or double-stranded) and the fragment size of the DNA extracted from buccal swab, blood, bone and tooth samples.
View Article and Find Full Text PDFRetin Cases Brief Rep
December 2024
Retinal Disorders and Ophthalmic Genetics Division, Stein Eye Institute, University of California Los Angeles, David Geffen School of Medicine at UCLA, Los Angeles, California, United States.
Purpose: To report a case of hydroxychloroquine (HCQ) retinopathy after long-term exposure in a 23-year-old male.
Methods: Multimodal imaging including fundus photography, fundus autofluorescence (FAF), spectral domain optical coherence tomography (SD-OCT), and en face OCT were performed, in addition to functional testing with full-field electroretinography (ERG) and Humphrey visual field (HVF).
Results: A 23-year-old man with a history of juvenile systemic lupus erythematosus and HCQ treatment for 13 years at a dosage of 200 mg/d (cumulative dose: 949 grams) presented to the retinal clinic (DS).
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