Cardiolipin deficiency in X-linked cardioskeletal myopathy and neutropenia (Barth syndrome, MIM 302060): a study in cultured skin fibroblasts.
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Monolysocardiolipin in cultured fibroblasts is a sensitive and specific marker for Barth SyndromeRole of calcium-independent phospholipase A2 in the pathogenesis of Barth syndromeCardiac and skeletal muscle defects in a mouse model of human Barth syndromeShotgun lipidomics of cardiolipin molecular species in lipid extracts of biological samplesAlterations in myocardial cardiolipin content and composition occur at the very earliest stages of diabetes: a shotgun lipidomics studyThe Role of Cardiolipin in Cardiovascular HealthMonolysocardiolipins accumulate in Barth syndrome but do not lead to enhanced apoptosisThe enzymatic function of tafazzinLPS impairs oxygen utilization in epithelia by triggering degradation of the mitochondrial enzyme Alcat1Cardiolipin fingerprinting of leukocytes by MALDI-TOF/MS as a screening tool for Barth syndromeDisorders of phospholipid metabolism: an emerging class of mitochondrial disease due to defects in nuclear genesA novel cardiolipin-remodeling pathway revealed by a gene encoding an endoplasmic reticulum-associated acyl-CoA:lysocardiolipin acyltransferase (ALCAT1) in mouseCardiolipin Regulates Mitophagy through the Protein Kinase C PathwayMitochondrial dysfunction and reduced prostaglandin synthesis in skeletal muscle of Group VIB Ca2+-independent phospholipase A2gamma-deficient mice.Direct MALDI-MS analysis of cardiolipin from rat organs sections.Distinct effects of tafazzin deletion in differentiated and undifferentiated mitochondria.Age-related decline in mitochondrial bioenergetics: does supercomplex destabilization determine lower oxidative capacity and higher superoxide production?The 3-methylglutaconic acidurias: what's new?A Drosophila model of Barth syndromeBarth syndrome without tetralinoleoyl cardiolipin deficiency: a possible ameliorated phenotype.Delineating the role of alterations in lipid metabolism to the pathogenesis of inherited skeletal and cardiac muscle disorders: Thematic Review Series: Genetics of Human Lipid Diseases.Barth syndrome, a human disorder of cardiolipin metabolism.Cardiolipin synthesis for the assembly of bacterial and mitochondrial membranes.Dysfunctional cardiac mitochondrial bioenergetic, lipidomic, and signaling in a murine model of Barth syndromeDiminished Exercise Capacity and Mitochondrial bc1 Complex Deficiency in Tafazzin-Knockdown MiceCardiolipin provides an essential activating platform for caspase-8 on mitochondria.Formation of molecular species of mitochondrial cardiolipin 2. A mathematical model of pattern formation by phospholipid transacylationAdvances in the understanding of Barth syndrome.The cellular and molecular mechanisms for neutropenia in Barth syndrome.A Novel Exonic Splicing Mutation in the TAZ (G4.5) Gene in a Case with Atypical Barth SyndromeTafazzin knockdown causes hypertrophy of neonatal ventricular myocytes.Remodeling of cardiolipin by phospholipid transacylation.Mis-sesnse mutations in Tafazzin (TAZ) that escort to mild clinical symptoms of Barth syndrome is owed to the minimal inhibitory effect of the mutations on the enzyme function: In-silico evidence.Clinicopathologic conference: Barth Syndrome.Tafazzin knockdown interrupts cell cycle progression in cultured neonatal ventricular fibroblasts.Cardiolipin content in mitochondria from cultured skin fibroblasts harboring mutations in the mitochondrial ATP6 gene
P2860
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P2860
Cardiolipin deficiency in X-linked cardioskeletal myopathy and neutropenia (Barth syndrome, MIM 302060): a study in cultured skin fibroblasts.
description
2002 nî lūn-bûn
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2002年の論文
@ja
2002年学术文章
@wuu
2002年学术文章
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2002年学术文章
@zh-cn
2002年学术文章
@zh-hans
2002年学术文章
@zh-my
2002年学术文章
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name
Cardiolipin deficiency in X-li ...... in cultured skin fibroblasts.
@en
Cardiolipin deficiency in X-li ...... in cultured skin fibroblasts.
@nl
type
label
Cardiolipin deficiency in X-li ...... in cultured skin fibroblasts.
@en
Cardiolipin deficiency in X-li ...... in cultured skin fibroblasts.
@nl
prefLabel
Cardiolipin deficiency in X-li ...... in cultured skin fibroblasts.
@en
Cardiolipin deficiency in X-li ...... in cultured skin fibroblasts.
@nl
P2093
P356
P1476
Cardiolipin deficiency in X-li ...... in cultured skin fibroblasts.
@en
P2093
Albert H Van Gennip
Barbara Plecko
Fredoen Valianpour
Henk Overmars
Kolja Becker
Peter G Barth
Peter Vreken
Rene Santer
P304
P356
10.1067/MPD.2002.129174
P407
P577
2002-11-01T00:00:00Z