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10.1007/s10545-015-9859-z http://scihub22266oqcxt.onion/10.1007/s10545-015-9859-z C4493943!4493943 !26016801     free     free     free Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=26016801 &cmd=llinks): Failed to open stream: HTTP request failed! HTTP/1.1 429 Too Many Requests in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 215 Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\26016801 .jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117       J+Inherit+Metab+Dis 2015 ; 38 (4 ): 655-80 Nephropedia Template TP {{tp|p=26016801 |t=2015. Mitochondrial transcript maturation and its disorders |pdf=|usr=}}{{26016801 }} gab.com Text Mitochondrial transcript maturation and its disorders JO: J Inherit Metab Dis http://www.kidney.de/pget.php?&tw=1&pmid=26016801 #FOAvip Mitochondrial respiratory chain deficiencies exhibit a wide spectrum of clinical presentations owing to defective mitochondrial energy production through oxidative phosphorylation. These defects can be caused by either mutations in the mitochondrial DNA (mtDNA) or mutations in nuclear genes coding for mitochondrially-targeted proteins. The underlying pathomechanisms can affect numerous pathways involved in mitochondrial biology including expression of mtDNA-encoded genes. Expression of the mitochondrial genes is extensively regulated at the post-transcriptional stage and entails nucleolytic cleavage of precursor RNAs, RNA nucleotide modifications, RNA polyadenylation, RNA quality and stability control. These processes ensure proper mitochondrial RNA (mtRNA) function, and are regulated by dedicated, nuclear-encoded enzymes. Recent growing evidence suggests that mutations in these nuclear genes, leading to incorrect maturation of RNAs, are a cause of human mitochondrial disease. Additionally, mutations in mtDNA-encoded genes may also affect RNA maturation and are frequently associated with human disease. We review the current knowledge on a subset of nuclear-encoded genes coding for proteins involved in mitochondrial RNA maturation, for which genetic variants impacting upon mitochondrial pathophysiology have been reported. Also, primary pathological mtDNA mutations with recognised effects upon RNA processing are described. Twit Text FOAVip Mitochondrial transcript maturation and its disorders ????J Inherit Metab Dis http://www.kidney.de/pget.php?&tw=1&pmid=26016801 #FOAvip Twit Text # Free Paper on # # # # # LINK http://www.kidney.de/pget.php?&tw=1&pmid=26016801 #FOAvip English Wikipedia <ref>{{Cite pmid|26016801 }}</ref> Mitochondrial transcript maturation and its disorders #MMPMID26016801 Van Haute L ; Pearce SF ; Powell CA ; D'Souza AR ; Nicholls TJ ; Minczuk M J Inherit Metab Dis 2015[Jul]; 38 (4 ): 655-80 PMID26016801 show ga Mitochondrial respiratory chain deficiencies exhibit a wide spectrum of clinical presentations owing to defective mitochondrial energy production through oxidative phosphorylation. These defects can be caused by either mutations in the mitochondrial DNA (mtDNA) or mutations in nuclear genes coding for mitochondrially-targeted proteins. The underlying pathomechanisms can affect numerous pathways involved in mitochondrial biology including expression of mtDNA-encoded genes. Expression of the mitochondrial genes is extensively regulated at the post-transcriptional stage and entails nucleolytic cleavage of precursor RNAs, RNA nucleotide modifications, RNA polyadenylation, RNA quality and stability control. These processes ensure proper mitochondrial RNA (mtRNA) function, and are regulated by dedicated, nuclear-encoded enzymes. Recent growing evidence suggests that mutations in these nuclear genes, leading to incorrect maturation of RNAs, are a cause of human mitochondrial disease. Additionally, mutations in mtDNA-encoded genes may also affect RNA maturation and are frequently associated with human disease. We review the current knowledge on a subset of nuclear-encoded genes coding for proteins involved in mitochondrial RNA maturation, for which genetic variants impacting upon mitochondrial pathophysiology have been reported. Also, primary pathological mtDNA mutations with recognised effects upon RNA processing are described. |DNA, Mitochondrial/metabolism [MESH] |Humans [MESH] |Mitochondria/*metabolism/*pathology [MESH] |Mitochondrial Diseases/genetics/*metabolism/*pathology [MESH]Mitochondrial transcript maturation and its disorders (epmc).pdf DeepDyve Pubget Overpricing