Mitochondrial respiratory chain supercomplexes are destabilized in Barth Syndrome patients
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Human trifunctional protein alpha links cardiolipin remodeling to beta-oxidationMitochondrial translation and beyond: processes implicated in combined oxidative phosphorylation deficienciesTIMMDC1/C3orf1 functions as a membrane-embedded mitochondrial complex I assembly factor through association with the MCIA complexC11orf83, a mitochondrial cardiolipin-binding protein involved in bc1 complex assembly and supercomplex stabilizationQIL1 is a novel mitochondrial protein required for MICOS complex stability and cristae morphologyThe cardiolipin transacylase, tafazzin, associates with two distinct respiratory components providing insight into Barth syndromeIdentification of the human mitochondrial linoleoyl-coenzyme A monolysocardiolipin acyltransferase (MLCL AT-1)Cardiolipin membrane domains in prokaryotes and eukaryotesCardiolipin and electron transport chain abnormalities in mouse brain tumor mitochondria: lipidomic evidence supporting the Warburg theory of cancerHuman CIA30 is involved in the early assembly of mitochondrial complex I and mutations in its gene cause diseaseAnalysis of the assembly profiles for mitochondrial- and nuclear-DNA-encoded subunits into complex ICombined defects in oxidative phosphorylation and fatty acid β-oxidation in mitochondrial diseaseBarth Syndrome: From Mitochondrial Dysfunctions Associated with Aberrant Production of Reactive Oxygen Species to Pluripotent Stem Cell StudiesMitochondria-controlled signaling mechanisms of brain protection in hypoxiaThe Role of Cardiolipin in Cardiovascular HealthSuccessful management of Barth syndrome: a systematic review highlighting the importance of a flexible and multidisciplinary approachSengers syndrome: six novel AGK mutations in seven new families and review of the phenotypic and mutational spectrum of 29 patientsRespiratory supercomplexes: plasticity and implicationsArrangement of electron transport chain components in bovine mitochondrial supercomplex I1III2IV1The translocator maintenance protein Tam41 is required for mitochondrial cardiolipin biosynthesis.Cardiolipin remodeling by TAZ/tafazzin is selectively required for the initiation of mitophagyMitochondria in heart failureLipids in the assembly of membrane proteins and organization of protein supercomplexes: implications for lipid-linked disordersDefining functional classes of Barth syndrome mutation in humansDisorders of phospholipid metabolism: an emerging class of mitochondrial disease due to defects in nuclear genesCellular functions of cardiolipin in yeastBiosynthesis, remodeling and turnover of mitochondrial cardiolipinCardiolipin remodeling: a regulatory hub for modulating cardiolipin metabolism and functionThe complexity of cardiolipin in health and diseaseOxidative stress, cardiolipin and mitochondrial dysfunction in nonalcoholic fatty liver diseaseGenetic variability of respiratory complex abundance, organization and activity in mouse brainA stabilizing factor for mitochondrial respiratory supercomplex assembly regulates energy metabolism in muscleProteomic and metabolomic analyses of mitochondrial complex I-deficient mouse model generated by spontaneous B2 short interspersed nuclear element (SINE) insertion into NADH dehydrogenase (ubiquinone) Fe-S protein 4 (Ndufs4) geneAnalysis of mouse models of cytochrome c oxidase deficiency owing to mutations in Sco2Cardiolipin, a critical determinant of mitochondrial carrier protein assembly and function.Supramolecular organization of the respiratory chain in Neurospora crassa mitochondriaDistinct effects of tafazzin deletion in differentiated and undifferentiated mitochondria.Mitochondria hyperfusion and elevated autophagic activity are key mechanisms for cellular bioenergetic preservation in centenarians.Age-related decline in mitochondrial bioenergetics: does supercomplex destabilization determine lower oxidative capacity and higher superoxide production?Coenzyme q and the respiratory chain: coenzyme q pool and mitochondrial supercomplexes
P2860
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P2860
Mitochondrial respiratory chain supercomplexes are destabilized in Barth Syndrome patients
description
2006 nî lūn-bûn
@nan
2006 թուականի Օգոստոսին հրատարակուած գիտական յօդուած
@hyw
2006 թվականի օգոստոսին հրատարակված գիտական հոդված
@hy
2006年の論文
@ja
2006年論文
@yue
2006年論文
@zh-hant
2006年論文
@zh-hk
2006年論文
@zh-mo
2006年論文
@zh-tw
2006年论文
@wuu
name
Mitochondrial respiratory chai ...... zed in Barth Syndrome patients
@ast
Mitochondrial respiratory chai ...... zed in Barth Syndrome patients
@en
Mitochondrial respiratory chai ...... zed in Barth Syndrome patients
@nl
type
label
Mitochondrial respiratory chai ...... zed in Barth Syndrome patients
@ast
Mitochondrial respiratory chai ...... zed in Barth Syndrome patients
@en
Mitochondrial respiratory chai ...... zed in Barth Syndrome patients
@nl
prefLabel
Mitochondrial respiratory chai ...... zed in Barth Syndrome patients
@ast
Mitochondrial respiratory chai ...... zed in Barth Syndrome patients
@en
Mitochondrial respiratory chai ...... zed in Barth Syndrome patients
@nl
P50
P921
P3181
P1476
Mitochondrial respiratory chai ...... zed in Barth Syndrome patients
@en
P2093
Michael T Ryan
P3181
P356
10.1016/J.JMB.2006.06.057
P407
P577
2006-08-18T00:00:00Z