Reactome | apo-UQCRFS1 binds LYRM7

apo-UQCRFS1 binds LYRM7

Stable Identifier

R-HSA-9866253

Type

Reaction [transition]

Species

Homo sapiens

Compartment

mitochondrial matrix

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 Sánchez, E, Lobo, T, Fox, JL, Zeviani, M, Winge, DR, Fernandez-Vizarra, E	Biochim Biophys Acta	2013
36757047	LYRM7-associated mitochondrial complex III deficiency with non-cavitating leukoencephalopathy and stroke-like episodes Alfattal, R, Alfarhan, M, Algaith, AM, Albash, B, Elshafie, RM, Alshammari, A, Alahmad, A, Dashti, F, Alsafi, R, Alsharhan, H	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, Kim, KS, Singh, A, Rouault, TA	Cell Metab	2017