Bcs1p, an AAA-family member, is a chaperone for the assembly of the cytochrome bc(1) complex.
about
The AAA+ superfamily of functionally diverse proteinsHeat shock protein 90 mediates protein-protein interactions between human aminoacyl-tRNA synthetasesMutations in TTC19 cause mitochondrial complex III deficiency and neurological impairment in humans and fliesSVIP is a novel VCP/p97-interacting protein whose expression causes cell vacuolation.GRACILE syndrome, a lethal metabolic disorder with iron overload, is caused by a point mutation in BCS1LAn in silico approach combined with in vivo experiments enables the identification of a new protein whose overexpression can compensate for specific respiratory defects in Saccharomyces cerevisiae.The LYR protein Mzm1 functions in the insertion of the Rieske Fe/S protein in yeast mitochondria.Late-stage maturation of the Rieske Fe/S protein: Mzm1 stabilizes Rip1 but does not facilitate its translocation by the AAA ATPase Bcs1The GRACILE mutation introduced into Bcs1l causes postnatal complex III deficiency: a viable mouse model for mitochondrial hepatopathyFunctional regulation of FEZ1 by the U-box-type ubiquitin ligase E4B contributes to neuritogenesisMetabolite profiles reveal energy failure and impaired beta-oxidation in liver of mice with complex III deficiency due to a BCS1L mutation.Mitochondrial protein import: recognition of internal import signals of BCS1 by the TOM complexMolecular clearance of ataxin-3 is regulated by a mammalian E4Impaired complex III assembly associated with BCS1L gene mutations in isolated mitochondrial encephalopathy.Cells lacking Rieske iron-sulfur protein have a reactive oxygen species-associated decrease in respiratory complexes I and IV.Yeast as a system for modeling mitochondrial disease mechanisms and discovering therapies.Biogenesis of the cytochrome bc(1) complex and role of assembly factors.The mitochondrial oxidative phosphorylation proteome of Chlamydomonas reinhardtii deduced from the Genome Sequencing Project.Dynamic subcompartmentalization of the mitochondrial inner membraneThe Cbp3-Cbp6 complex coordinates cytochrome b synthesis with bc(1) complex assembly in yeast mitochondriaThe two rotor components of yeast mitochondrial ATP synthase are mechanically coupled by subunit delta.Evidence that the assembly of the yeast cytochrome bc1 complex involves the formation of a large core structure in the inner mitochondrial membraneThe power of yeast to model diseases of the powerhouse of the cell.Modulation of the respiratory supercomplexes in yeast: enhanced formation of cytochrome oxidase increases the stability and abundance of respiratory supercomplexes.Diverse functions of mitochondrial AAA+ proteins: protein activation, disaggregation, and degradation.Mitochondrial disorders caused by mutations in respiratory chain assembly factors.Hair curvature: a natural dialectic and review.Nuclear gene mutations as the cause of mitochondrial complex III deficiencyMutations in mitochondrial complex III uniquely affect complex I in Caenorhabditis elegans.Toxic response caused by a misfolding variant of the mitochondrial protein short-chain acyl-CoA dehydrogenase.An animal model manifesting neurodegeneration and obesity.The Rieske Iron-Sulfur Protein: Import and Assembly into the Cytochrome bc(1) Complex of Yeast Mitochondria.Mitochondrial complex III Rieske Fe-S protein processing and assembly.Respiratory chain enzyme deficiency induces mitochondrial location of actin-binding gelsolin to modulate the oligomerization of VDAC complexes and cell survival.The cytochrome bc1 and cytochrome c oxidase complexes associate to form a single supracomplex in yeast mitochondria.Comparison of normal human skin gene expression using cDNA microarrays.Assembly of mammalian oxidative phosphorylation complexes I-V and supercomplexesClinical and diagnostic characteristics of complex III deficiency due to mutations in the BCS1L gene
P2860
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P2860
Bcs1p, an AAA-family member, is a chaperone for the assembly of the cytochrome bc(1) complex.
description
1999 nî lūn-bûn
@nan
1999 թուականի Հոկտեմբերին հրատարակուած գիտական յօդուած
@hyw
1999 թվականի հոտեմբերին հրատարակված գիտական հոդված
@hy
1999年の論文
@ja
1999年論文
@yue
1999年論文
@zh-hant
1999年論文
@zh-hk
1999年論文
@zh-mo
1999年論文
@zh-tw
1999年论文
@wuu
name
Bcs1p, an AAA-family member, i ...... the cytochrome bc(1) complex.
@ast
Bcs1p, an AAA-family member, i ...... the cytochrome bc(1) complex.
@en
Bcs1p, an AAA-family member, i ...... the cytochrome bc(1) complex.
@nl
type
label
Bcs1p, an AAA-family member, i ...... the cytochrome bc(1) complex.
@ast
Bcs1p, an AAA-family member, i ...... the cytochrome bc(1) complex.
@en
Bcs1p, an AAA-family member, i ...... the cytochrome bc(1) complex.
@nl
prefLabel
Bcs1p, an AAA-family member, i ...... the cytochrome bc(1) complex.
@ast
Bcs1p, an AAA-family member, i ...... the cytochrome bc(1) complex.
@en
Bcs1p, an AAA-family member, i ...... the cytochrome bc(1) complex.
@nl
P2093
P356
P1433
P1476
Bcs1p, an AAA-family member, i ...... f the cytochrome bc(1) complex
@en
P2093
P304
P356
10.1093/EMBOJ/18.19.5226
P407
P50
P577
1999-10-01T00:00:00Z