Results 81 to 90 of about 1,622,122 (202)

Aberrant TMPRSS6‐Protease Regulation of Disease Mutant HCN4–KCNE1 Channel Complex Depends on the KCNE1‐G38S Polymorphism

open access: yesArchiv der Pharmazie, Volume 359, Issue 6, June 2026.
TMPRSS6‐mediated cleavage of the HCN4–KCNE1 channel complex may modulate disease phenotype in a KCNE1 genotype‐dependent manner. ABSTRACT The sinoatrial node pacemaker channel HCN4 plays a central role in cardiac automaticity, and disease‐associated variants can predispose to atrial arrhythmias.
David Linhoff   +12 more
wiley   +1 more source

Brief von Michael Barth an Kurt Rothschild

open access: yes
BRIEF VON MICHAEL BARTH AN KURT ROTHSCHILD Brief von Michael Barth an Kurt Rothschild ([1]
Barth, Michael
core   +3 more sources

Johann Christoph Maiers ... Beschreibung von Venedig : Mit Grundrissen und Kupfern

open access: yes
Vorlageform des Erscheinungsvermerks: Leipzig, bei Johann Ambrosius Barth 1795.Titelkupfer, Kt. (Kupferst.
Maier, Johann Christoph
core   +1 more source

AAV9-TAZ Gene Replacement Ameliorates Cardiac TMT Proteomic Profiles in a Mouse Model of Barth Syndrome

open access: yesMolecular Therapy: Methods & Clinical Development, 2019
Barth syndrome (BTHS) is a rare mitochondrial disease that causes severe cardiomyopathy and has no disease-modifying therapy. It is caused by recessive mutations in the gene tafazzin (TAZ), which encodes tafazzin—an acyltransferase that remodels the ...
Silveli Suzuki-Hatano   +6 more
doaj   +1 more source

Phospholipid abnormalities in children with Barth syndrome

open access: yesJournal of the American College of Cardiology, 2003
We sought to identify characteristic lipid abnormalities in patients with Barth syndrome (BTHS) and to correlate the lipid profile to phenotype and genotype.Barth syndrome typically includes cardiomyopathy, skeletal myopathy, neutropenia, growth retardation, and 3-methylglutaconic aciduria, and it is commonly associated with mutations in the tafazzin ...
Schlame, Michael   +8 more
openaire   +3 more sources

Philoikos zur Beförderung häuslicher Tugend und Glückseligkeit

open access: yes
Autopsie nach Exemplar der ULB Sachsen-AnhaltVorlageform des Erscheinungsvermerks: Leipzig, bei Johann Ambrosius Barth ...
Pischon, Johann Karl
core   +1 more source

A novel intronic splice site tafazzin gene mutation detected prenatally in a family with Barth syndrome

open access: yesBalkan Journal of Medical Genetics, 2016
Barth syndrome (BTHS) is a rare X-linked disease characterized by dilated cardiomyopathy, proximal skeletal myopathy and cyclic neutropenia. It is caused by various mutations in the tafazzin (TAZ) gene located on Xq28 that results in remodeling of ...
Bakšienė M   +5 more
doaj   +1 more source

Emerging roles of pyruvate dehydrogenase phosphatase 1: a key player in metabolic health

open access: yesFrontiers in Physiology
Pyruvate dehydrogenase phosphatase (PDP), a structurally conserved member of the protein phosphatase C family (PP2C) of proteins, is a key regulatory enzyme responsible for reactivation of the mitochondrial gate-keeper, pyruvate dehydrogenase (PDH ...
Vikalp Kumar, Miriam L. Greenberg
doaj   +1 more source

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