apo-UQCRFS1 binds LYRM7

Stable Identifier
R-HSA-9866253
Type
Reaction [transition]
Species
Homo sapiens
Compartment
ReviewStatus
5/5
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After its synthesis in the cytosol and via to the mitochondrial matrix following the TOM and TIM23 pathway, the Complex III subunit UQCRFS1 (Rieske protein) binds to and is stabilized by the chaperone LYRM7 (MZM1L) (Sanchez et al., 2012; Maio et al., 2017; Fernandez-Vizarra & Zeviani, 2018). Defects in LYRM7 lead to Complex III deficiency, manifesting in early-onset leukencephalopathy (reviewed in Alfattal et al., 2023).
Literature References
PubMed ID Title Journal Year
29243944 Mitochondrial complex III Rieske Fe-S protein processing and assembly

Fernandez-Vizarra, E, Zeviani, M

Cell Cycle 2018
23168492 LYRM7/MZM1L is a UQCRFS1 chaperone involved in the last steps of mitochondrial Complex III assembly in human cells

Lobo, T, Winge, DR, Fernandez-Vizarra, E, Fox, JL, Sánchez, E, Zeviani, M

Biochim Biophys Acta 2013
36757047 LYRM7-associated mitochondrial complex III deficiency with non-cavitating leukoencephalopathy and stroke-like episodes

Alsafi, R, Alahmad, A, Alfarhan, M, Dashti, F, Alshammari, A, Alfattal, R, Algaith, AM, Alsharhan, H, Elshafie, RM, Albash, B

Am J Med Genet A 2023
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
Participants
Participates
Catalyst Activity

protein folding chaperone activity of LYRM7 [mitochondrial matrix]

Orthologous Events
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