There are two 5′UTRs that are found preceding either the ORF encoding ND4 or the ORF for ATPase6. Both of these ORFs constitute the downstream part of bicistronic transcripts RNA14 and RNA7, respectively, and hence the 5′UTR is in fact the out of frame sequence encoding each of the ...
ATPase activity of LONP1 was required for CLPX solubilization (Supplementary Fig.5g), and LONP1 S855A interacted with solubilized CLPX (Supplementary Fig.5h). Unlike the case with OXA1L, however, we did not observe synergist activity when LONP1 and mtHSP70 were added to the same reaction. ...
Tim14, a novel key component of the import motor of the TIM23 protein translocase of mitochondria. EMBO J 2003b; 22: 4945–4956. Article CAS Google Scholar Neupert W . A perspective on transport of proteins into mitochondria: a myriad of open questions. J Mol Biol 2015; 427: 1135–...
in which microscopic analysis and conventional OXPHOS activity (complex I-IV) tend to be unremarkable, and the evaluation of ATPase activity is likely to appear normal as well, validating the pathogenicity of rare or novel variants poses a diagnostic challenge. Especially when these variants are ho...