CLPP Coordinates Mitoribosome Assembly - Maiti, Priyanka
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AAA+ enzymes utilizes the energy of ATP binding and hydrolysis to drive many biological processes. ClpXP is a highly conserved AAA+ protease, present in bacteria and found in the mitochondria of eukaryotic cells. ClpXP consists of two proteins, a AAA+ ATPase ClpX and a peptidase ClpP. Unfortunately, very little is known about the role of ClpXP in mammalian mitochondria, despite the first patients carrying a homozygous loss-of-function mutation in Clpp, presenting Perrault syndrome have been identified. My PhD studies focused on the understanding the role of CLPP in mammals in health and…mehr

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Produktbeschreibung
AAA+ enzymes utilizes the energy of ATP binding and hydrolysis to drive many biological processes. ClpXP is a highly conserved AAA+ protease, present in bacteria and found in the mitochondria of eukaryotic cells. ClpXP consists of two proteins, a AAA+ ATPase ClpX and a peptidase ClpP. Unfortunately, very little is known about the role of ClpXP in mammalian mitochondria, despite the first patients carrying a homozygous loss-of-function mutation in Clpp, presenting Perrault syndrome have been identified. My PhD studies focused on the understanding the role of CLPP in mammals in health and disease. For this purpose, I created and characterized Clpp knockout (KO) mice. CLPP deficient mice faithfully replicate the phenotypes observed in human patients. We observed a decrease in mitochondrial translation, suggesting a direct or indirect role of CLPP in mitochondrial translation. We determined that the mitoribosomal assembly defect is a consequence of the accumulation of ERAL1, a novel ClpXP substrate. Our data suggest that the timely removal of ERAL1 from the small ribosomal subunit is essential for the efficient maturation of the mitoribosome and a normal rate of mito-translation.
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Autorenporträt
After completing her engineering in biotechnology in India, Priyanka moved to USA to obtain her MS in microbiology. She walked into the remarkable field of mitochondrial biology during her PhD studies in Germany. Currently she is pursuing her postdoctoral training in USA. Besides science, she loves traveling, meditation, music, table tennis & chess