Maturation of frataxin within mammalian and yeast mitochondria: one-step processing by matrix processing peptidase
about
In vivo maturation of human frataxinCrystal structure of human frataxinCrystal structure of Escherichia coli CyaY protein reveals a previously unidentified fold for the evolutionarily conserved frataxin familyFriedreich’s Ataxia Variants I154F and W155R Diminish Frataxin-Based Activation of the Iron–Sulfur Cluster Assembly ComplexA GTP:AMP phosphotransferase, Adk2p, in Saccharomyces cerevisiae. Role of the C terminus in protein folding/stabilization, thermal tolerance, and enzymatic activity.J-domain protein, Jac1p, of yeast mitochondria required for iron homeostasis and activity of Fe-S cluster proteins.Two-step processing of human frataxin by mitochondrial processing peptidase. Precursor and intermediate forms are cleaved at different ratesFriedreich ataxia: from GAA triplet-repeat expansion to frataxin deficiency.Friedreich ataxia: molecular mechanisms, redox considerations, and therapeutic opportunities.Frataxin and mitochondrial FeS cluster biogenesis.Intracellular iron transport and storage: from molecular mechanisms to health implications.Molecular basis of Friedreich ataxia.Stem cells from wildtype and Friedreich's ataxia mice present similar neuroprotective properties in dorsal root ganglia cells.Altered zinc transport disrupts mitochondrial protein processing/import in fragile X-associated tremor/ataxia syndrome.Key players and their role during mitochondrial iron-sulfur cluster biosynthesisMutations in the substrate binding glycine-rich loop of the mitochondrial processing peptidase-α protein (PMPCA) cause a severe mitochondrial disease.Advancements in the pathophysiology of Friedreich's Ataxia and new prospects for treatments.Metal complexes and metalloproteases: targeting conformational diseases.Selected missense mutations impair frataxin processing in Friedreich ataxiaGTP in the mitochondrial matrix plays a crucial role in organellar iron homoeostasisDual role of the mitochondrial protein frataxin in astrocytic tumors.Functional and molecular characterization of the frataxin homolog from Arabidopsis thaliana.
P2860
Q24304209-29CDA4A4-8670-4C21-9573-5674D6DBEE62Q27625397-3EE9614B-60BE-4F79-8263-7B6538694929Q27625486-01CD0788-85CD-40E1-9745-86D80821824EQ27670432-39D4D027-674E-43BD-82DF-B421C29954ADQ27931823-E1BCB425-67E2-4375-9F56-9694ADE50E64Q27933878-E2C5C760-4BA8-4390-A709-AEF1DF1D1F10Q28140463-9D1EE22E-5A41-4EE0-B4D5-14B1DA545525Q28200651-40D61F14-89A6-492B-9A57-A941B59AC652Q34076650-0B6B9BD5-DCE3-4184-AB6C-E1CB919E26A7Q34094277-B90588A1-7A13-4F68-9B8D-A0F7C6FE7A58Q34100135-99E17AC6-DF0A-4F80-A2E0-5FFA23F17BECQ34464836-DA87FD55-C668-4722-9E1F-4B91808977AAQ34720833-091CF49E-9AF7-4BE3-8E5F-DEF41F9140FAQ35090105-FF5818F0-254F-4F6B-9A79-469E95E77068Q36005200-FF78FEEF-DBCE-46CF-97A8-56A6ABF481C6Q36859413-DF8D8FBA-BBE8-4060-8600-2AE90FE4A5E0Q36863208-5EAD6908-0E16-4AE3-A8EA-3435600C4E6BQ38215608-1C7D44C4-E247-418B-A390-18D4B67C18C2Q38622735-21192FE6-ACC8-4825-B7E0-9D8A80F31C6DQ39087163-8A6B83BB-ACE6-4EFE-B33D-F56AA003F510Q39485587-B21EABD8-4762-430A-86B8-3A3C848875FAQ47914805-ED68C5B1-BB09-41DF-BED2-08D322A17A7B
P2860
Maturation of frataxin within mammalian and yeast mitochondria: one-step processing by matrix processing peptidase
description
1999 nî lūn-bûn
@nan
1999 թուականի Նոյեմբերին հրատարակուած գիտական յօդուած
@hyw
1999 թվականի նոյեմբերին 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
Maturation of frataxin within ...... by matrix processing peptidase
@ast
Maturation of frataxin within ...... by matrix processing peptidase
@en
Maturation of frataxin within ...... by matrix processing peptidase
@nl
type
label
Maturation of frataxin within ...... by matrix processing peptidase
@ast
Maturation of frataxin within ...... by matrix processing peptidase
@en
Maturation of frataxin within ...... by matrix processing peptidase
@nl
prefLabel
Maturation of frataxin within ...... by matrix processing peptidase
@ast
Maturation of frataxin within ...... by matrix processing peptidase
@en
Maturation of frataxin within ...... by matrix processing peptidase
@nl
P2093
P3181
P356
P1476
Maturation of frataxin within ...... by matrix processing peptidase
@en
P2093
P304
P3181
P356
10.1093/HMG/8.12.2255
P407
P577
1999-11-01T00:00:00Z