Abstract Background Barth syndrome (BTHS) is an inborn error of phospholipid metabolism caused by pathogenic variants in the TAFAZZIN gene locus on a chromosome X. encodes transacylase enzyme, tafazzin, that promotes cardiolipin (CL) remodeling. Deficiency tafazzin activity BTHS results accumulation monolysocardiolipin (MLCL) and decreased abundance mature CL, making increased ratio MLCL to CL ...

In the title compound, [VClO(C(12)H(12)N(2))(2)]Cl·C(2)H(5)OH·H(2)O, the V(IV) atom is six-coordinated in a distorted octa-hedral geometry by four N atoms from two 4,4'-dimethyl-2,2'-bipyridine ligands, one O atom and one Cl atom. In the crystal, O-H⋯Cl, C-H⋯O and C-H⋯Cl hydrogen bonds and π-π contacts between the pyridine rings [centroid-centroid distances = 3.7236 (17) and 3.6026 (19) Å] stab...

BACKGROUND The molecular mechanism of K-Cl cotransport (KCC) consists of at least 4 isoforms, KCC 1, 2, 3, and 4 which, in multiple combinations, exist in most cells, including erythrocytes and neuronal cells. METHODS We utilized reverse-transcriptase-polymerase chain reaction (RT-PCR) and ion flux studies to characterize KCC activity in an immortalized in vitro cell model for fibrous astrocy...

We reported previously [Kataoka, Muroi, Ohkuma, Waritani, Magae, Takatsuki, Kondo, Yamasaki and Nagai (1995) FEBS Lett. 359, 53-59] that prodigiosin 25-C (one of the red pigments of the prodigiosin group produced by micro-organisms like Streptomyces and Serratia) uncoupled vacuolar H+-ATPase, inhibited vacuolar acidification and affected glycoprotein processing. In the present study we show tha...

Calu-3 cells secrete HCO(3)(-) in response to cAMP agonists but can be stimulated to secrete Cl(-) with K(+) channel activating agonists. Microelectrode and impedance analysis experiments were performed to obtain a better understanding of the conductances and driving forces involved in these different modes of anion secretion in Calu-3 cells. Microelectrode studies revealed apical and basolater...