Transfer of Fe-S clusters to SDHB

Stable Identifier
Reaction [uncertain]
Homo sapiens
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After translation in cytosol and translocation to the mitochondrial inner membrane SDH subunits C and D (SDHC, SDHD), together with the heme b cofactor, bind to the assembled soluble SDHA:SDHB construct to form the fully functional SDH complex in the mitochondrial membrane. Although a full structure of the porcine complex exists (PDB 1ZOY) no details are known about the last steps of the assembly. However, successful assembly depends on the presence of both the SDHC heme b cofactor and the C-terminal tail of the SDHA subunit. (Lemarie & Grimm, 2009; Kim et al., 2012; reviewed in Van Vranken et al., 2014; Signes & Fernandez-Vizarra, 2018).
Literature References
PubMed ID Title Journal Year
24954417 The LYR factors SDHAF1 and SDHAF3 mediate maturation of the iron-sulfur subunit of succinate dehydrogenase

Yu, W, Thummel, CS, Rutter, J, Brockmann, K, Na, U, Winge, DR, Bricker, DK, Cox, J

Cell Metab 2014
26749241 Disease-Causing SDHAF1 Mutations Impair Transfer of Fe-S Clusters to SDHB

Carrozzo, R, Maio, N, Martinelli, D, Rizza, T, Verrigni, D, Singh, A, Ghezzi, D, Rouault, TA, Bertini, E, Zeviani, M

Cell Metab 2016
28380382 A Single Adaptable Cochaperone-Scaffold Complex Delivers Nascent Iron-Sulfur Clusters to Mammalian Respiratory Chain Complexes I-III

Maio, N, Singh, A, Kim, KS, Rouault, TA

Cell Metab 2017
30030361 Assembly of mammalian oxidative phosphorylation complexes I-V and supercomplexes

Signes, A, Fernandez-Vizarra, E

Essays Biochem 2018
25488574 Protein-mediated assembly of succinate dehydrogenase and its cofactors

Rutter, J, Van Vranken, JG, Na, U, Winge, DR

Crit Rev Biochem Mol Biol 2015
Catalyst Activity

iron chaperone activity of 2 Iron:FXN [mitochondrial matrix]

Orthologous Events
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