Results 101 to 110 of about 2,732 (148)

A murine model of Barth syndrome recapitulates human cardiac and skeletal muscle phenotypes

Disease Models & Mechanisms
Erika Yazawa, Erin M. Keating, Suya Wang, Mason E. Sweat, Qing Ma, Yang Xu, Michael Schlame, William T. Pu   +7 more
doaj   +1 more source

Saturated cardiolipins are potent disruptors of inner mitochondrial membrane structure and function. [PDF]

J Biol Chem
Venkatraman K, Milshteyn D, Sarto C, Kocharian E, Sakurai CM, Armando AM, Wong AM, Dennis EA, Fang X, Lee CT, Budin I.   +10 more
europepmc   +1 more source

Granulopoietic Dysregulation in a Patient-Tailored Mouse Model of Barth Syndrome. [PDF]

Stem Cell Rev Rep
Sierra Potchanant EA, Capitano ML, Edwards DM, Ramdas B, Cooper S, Ropa J, Pay SL, Sheth A, Snider PL, Vernon HJ, Tran NT, Kapur R, Conway SJ.   +12 more
europepmc   +1 more source

Elamipretide in pediatric Barth syndrome: from heart failure to school return. [PDF]

Orphanet J Rare Dis
Amedro P, Andrianoely M, Gohier P, Leroux J, Aladjidi N, Lagorce S, Daccord M, Audie M, Botte A, Nubret K, Richard P, Roche S, Lamireau D, Tetard C, Trimouille A, Desir A, Mesli S, Donadieu J, Gotchac J.   +18 more
europepmc   +1 more source

Insights into Neutrophil Dysfunction in Inherited Metabolic Disorders. [PDF]

J Innate Immun
Wolf A, Montanelli G, Mathis D, Felser A, Laemmle A, Nuoffer JM, Gautschi M, Bourquin C, Stojkov D.   +8 more
europepmc   +1 more source

Running rich: how excess fatty acid oxidation drains the cardiac engine. [PDF]

J Clin Invest
Claypool SM, Koehler CM.
europepmc   +1 more source

De-succinylation-induced accumulation of TRMT10C in the nucleus plays a detrimental role in coronary microembolization via its m1A modification function. [PDF]

Int J Biol Sci
Hu CK, Huang WZ, He L, Chang C, Ren YL, Dai RX, Wu Q, Su Q.   +7 more
europepmc   +1 more source

Disturbed mitochondrial maturation in cardiolipin remodeling-deficient cardiomyocytes. [PDF]

iScience
Senoo N, Sheridan MS, Wohlfarter Y, Primrose MT, Tampakakis E, Keller MA, Claypool SM.   +6 more
europepmc   +1 more source

Peroxisomal catalase and plasmalogen biosynthesis protect from oxidative stress in Barth syndrome cardiomyopathy. [PDF]

Basic Res Cardiol
Kajese E, Hachmann M, Ermer KJ, Erk M, Alhasaan L, Kohlhaas M, Bömmel H, Berberich L, Carlein C, Eberl H, Streckfuß-Bömeke K, Roma LP, Ergün S, Maack C, Karnati S, Dudek J.   +15 more
europepmc   +1 more source

Letter to the Editor: CRISPR-based gene editing for cardiac protection in Barth syndrome. [PDF]

Ann Med Surg (Lond)
Abedin ZU, Waggan AI, Khan E, Suleman MU, Tabassum SN.   +4 more
europepmc   +1 more source