The V-type H+ ATPase: molecular structure and function,physiological roles and regulation

KW Beyenbach, H Wieczorek - Journal of Experimental …, 2006 - journals.biologists.com
It was nearly 30 years before the V-type H+ ATPase was admitted to the small circle of bona
fide transport ATPases alongside F-type and P-type ATPases. The V-type H+ ATPase is an …

Supporting sensory transduction: cochlear fluid homeostasis and the endocochlear potential

P Wangemann - The Journal of physiology, 2006 - Wiley Online Library
The exquisite sensitivity of the cochlea, which mediates the transduction of sound waves into
nerve impulses, depends on the endocochlear potential and requires a highly specialized …

Mutations in the gene encoding B1 subunit of H+-ATPase cause renal tubular acidosis with sensorineural deafness

FE Karet, KE Finberg, RD Nelson, A Nayir, H Mocan… - Nature …, 1999 - nature.com
H+-ATPases are ubiquitous in nature; V-ATPases pump protons against an electrochemical
gradient, whereas F-ATPases reverse the process, synthesizing ATP. We demonstrate here …

[HTML][HTML] Mutations in the gene encoding tight junction claudin-14 cause autosomal recessive deafness DFNB29

ER Wilcox, QL Burton, S Naz, S Riazuddin, TN Smith… - Cell, 2001 - cell.com
Tight junctions in the cochlear duct are thought to compartmentalize endolymph and provide
structural support for the auditory neuroepithelium. The claudin family of genes is known to …

Renal Vacuolar H+-ATPase

CA Wagner, KE Finberg, S Breton… - Physiological …, 2004 - journals.physiology.org
Vacuolar H+-ATPases are ubiquitous multisubunit complexes mediating the ATP-dependent
transport of protons. In addition to their role in acidifying the lumen of various intracellular …

Novel ATP6V1B1 and ATP6V0A4 mutations in autosomal recessive distal renal tubular acidosis with new evidence for hearing loss

EH Stover, KJ Borthwick, C Bavalia, N Eady… - Journal of medical …, 2002 - jmg.bmj.com
Autosomal recessive distal renal tubular acidosis (rdRTA) is characterised by severe
hyperchloraemic metabolic acidosis in childhood, hypokalaemia, decreased urinary calcium …

The pathophysiology of distal renal tubular acidosis

CA Wagner, R Unwin, SC Lopez-Garcia… - Nature Reviews …, 2023 - nature.com
The kidneys have a central role in the control of acid–base homeostasis owing to
bicarbonate reabsorption and production of ammonia and ammonium in the proximal tubule …

Mechanisms of otoconia and otolith development

YW Lundberg, Y Xu, KD Thiessen… - Developmental …, 2015 - Wiley Online Library
Background: Otoconia are bio‐crystals that couple mechanic forces to the sensory hair cells
in the utricle and saccule, a process essential for us to sense linear acceleration and gravity …

Expression pattern of the mouse ortholog of the Pendred's syndrome gene (Pds) suggests a key role for pendrin in the inner ear

LA Everett, H Morsli, DK Wu… - Proceedings of the …, 1999 - National Acad Sciences
Pendred's syndrome is an autosomal-recessive disorder characterized by deafness and
goiter. After our recent identification of the human gene mutated in Pendred's syndrome …

The H+-ATPase (V-ATPase): from proton pump to signaling complex in health and disease

AF Eaton, M Merkulova… - American Journal of …, 2021 - journals.physiology.org
A primary function of the H+-ATPase (or V-ATPase) is to create an electrochemical proton
gradient across eukaryotic cell membranes, which energizes fundamental cellular …