Reactome | Transfer of Fe-S clusters to SDHB

Transfer of Fe-S clusters to SDHB

Stable Identifier

R-HSA-9854984

Type

Reaction [uncertain]

Species

Homo sapiens

Compartment

mitochondrial matrix

ReviewStatus

5/5

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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