Knockout Mouse Studies Show That Mitochondrial CLPP Peptidase and CLPX Unfoldase Act in Matrix Condensates near IMM as Fast Stress Response in Protein Assemblies for Transcript Processing Translation and Heme Production.
The ubiquitous mitochondrial protein unfoldase CLPX regulates erythroid heme synthesis by control of iron utilization and heme synthesis enzyme activation and turnover.
Loss of mitochondrial peptidase Clpp leads to infertility hearing loss plus growth retardation via accumulation of CLPX mtDNA and inflammatory factors.
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