Results 61 to 70 of about 2,665 (161)
Substrate for Thyroid Hormone Synthesis: Biochemistry, Evolution, and Physiology
Two proposed sequence of events for thyroxine formation in patients expressing mutant thyroglobulin. (A) (1) TSH stimulation drives expression of misfolded TG. (2) Unremitting ER stress. (3) Stress‐induced cell death. (4) Dead thyrocytes release their contents. (5) The contents become iodinated. (6) Forming thyroxine.
Crystal Young, Peter Arvan
wiley +1 more source
KCC3a, a Strong Candidate Pathway for K+ Loss in Alkalemia
Loss-of-function mutations in the human potassium chloride cotransporter-3 (KCC3) cause a hereditary motor sensory neuropathy associated with agenesis of the corpus callosum. While recapitulating the neuropathy, KCC3-knockout mice also exhibit high blood
Mohammed Zubaerul Ferdaus +3 more
doaj +1 more source
Sensorineural hearing loss (SNHL) occurs in 1.5 billion globally, primary driven by damage of cochlear hair cells and spiral ganglion neurons. This review systematically examines current therapeutic strategies for sensorineural hearing loss, including traditional drug and physical therapies as well as emerging stem cell and gene therapies, providing a ...
Ruirui Chen +3 more
wiley +1 more source
ABSTRACT Aim Biallelic inactivating WDR72 variants are linked to distal renal tubular acidosis (dRTA), nephrocalcinosis, and amelogenesis imperfecta. The kidney shows high WDR72 expression; its precise localization and function remain unclear. WDR72 is a member of the WD40 repeat domain protein family—a large group of scaffold proteins involved in ...
Hannah Auwerx +4 more
wiley +1 more source
The HSP70 co-chaperone DNAJC14 targets misfolded pendrin for unconventional protein secretion
Mutations in pendrin, a plasma membrane transporter, lead to Pendred syndrome, which is associated with hearing loss. Here, Jung et al. show that cell-surface expression of a mutated form of pendrin can be restored by blocking ER-to-Golgi traffic and ...
Jinsei Jung +7 more
doaj +1 more source
Regulation of extracellular fluid volume and blood pressure by pendrin.
Na(+) is commonly designed as the culprit of salt-sensitive hypertension but several studies suggest that abnormal Cl(-) transport is in fact the triggering mechanism. This review focuses on the regulation of blood pressure (BP) by pendrin, an apical Cl(-
Giovanna Valenti +11 more
core +1 more source
Abstract figure legend Fick's law: JCO2=PM*,CO2±hAQP5·([CO2]os±bCA−[CO2]is±hCAII)TransmembraneCO2gradient${J}_{{\mathrm{CO}}_{2}}=\underset{\pm \mathrm{hAQP}5}{\underset{}{{P}_{\mathrm{M}^\ast ,{\mathrm{CO}}_{2}}}}\, \cdot \underset{\mathrm{Transmembrane}\ {\mathrm{CO}}_{2}\ \mathrm{gradient}}{\underset{}{(\underset{\pm \mathrm{bCA}}{\underset ...
Deng‐Ke Wang +2 more
wiley +1 more source
Effects of cAMP and CFTR modulation on apical fluid pH in human airway Calu‐3 cells
Abstract The airway epithelium serves as the first line of defense against inhaled insults present in the external environment by acting as a physical barrier and through host defense mechanisms. Proper maintenance of these host defense mechanisms relies on the regulation of airway surface liquid (ASL) composition and properties, a process that is ...
Jenny P. Nguyen +2 more
wiley +1 more source
Background: Probenecid is a uricosuric agent that in addition to exerting a positive ionotropic effect in the heart, blocks the ATP transporter Pannexin 1 and inhibits the Cl-/HCO3- exchanger, pendrin.
Sharon Barone +9 more
doaj +1 more source
Viewing Cortical Collecting Duct Function Through Phenotype-guided Single-Tubule Proteomics
The revolution of the omics technologies has enabled profiling of the molecules of any sample. However, the heterogeneity of the kidney with highly specialized nephron segments like the cortical collecting duct (CCD) poses a challenge regarding ...
Nina Himmerkus +8 more
doaj +1 more source

