Aliases for LYRM7 Gene
External Ids for LYRM7 Gene
Previous Symbols for LYRM7 Gene
Inner mitochondrial membrane complex III (CIII) is the main enzyme complex in the mitochondrial respiratory chain, and Rieske Fe-S protein (UQCRFS1) is the last catalytic subunit added to the complex. The protein encoded by this gene is a nuclear-encoded mitochondrial matrix protein that stabilizes UQCRFS1 and chaperones it to the CIII complex. Defects in this gene are a cause of mitochondrial complex III deficiency, nuclear type 8. Three transcript variants encoding two different isoforms have been found for this gene. [provided by RefSeq, Jun 2014]
GeneCards Summary for LYRM7 Gene
LYRM7 (LYR Motif Containing 7) is a Protein Coding gene. Diseases associated with LYRM7 include mitochondrial complex iii deficiency, nuclear type 8 and mitochondrial complex iii deficiency.
UniProtKB/Swiss-Prot for LYRM7 Gene
Assembly factor required for Rieske Fe-S protein UQCRFS1 incorporation into the cytochrome b-c1 (CIII) complex. Functions as a chaperone, binding to this subunit within the mitochondrial matrix and stabilizing it prior to its translocation and insertion into the late CIII dimeric intermediate within the mitochondrial inner membrane.