نتایج جستجو برای: trna mitochondrial mutation repeated pregnancy loss

تعداد نتایج: 1060873  

Journal: :Proceedings of the National Academy of Sciences of the United States of America 2007
Alexandre Ambrogelly Sarath Gundllapalli Stephanie Herring Carla Polycarpo Carina Frauer Dieter Söll

Pyrrolysine (Pyl), the 22nd naturally encoded amino acid, gets acylated to its distinctive UAG suppressor tRNA(Pyl) by the cognate pyrrolysyl-tRNA synthetase (PylRS). Here we determine the RNA elements required for recognition and aminoacylation of tRNA(Pyl) in vivo by using the Pyl analog N-epsilon-cyclopentyloxycarbonyl-l-lysine. Forty-two Methanosarcina barkeri tRNA(Pyl) variants were tested...

2018
Urszula Lechowicz Agnieszka Pollak Agnieszka Frączak Małgorzata Rydzanicz Piotr Stawiński Artur Lorens Piotr H. Skarżyński Henryk Skarżyński Rafał Płoski Monika Ołdak

Interruptions in the activity of mitochondria induced by mutations in the mitochondrial genome (mtDNA) can be the source of numerous diseases including hearing loss (HL). One of the mitochondrial variants responsible for HL is the m.7511T>C mutation located in the mitochondrially encoded tRNA serine 1 (UCN) gene. Next‑generation sequencing was used to search for the HL mutations in the whole mt...

Journal: :Pediatrics 2004
Johanna Uusimaa Saara Finnilä Anne M Remes Heikki Rantala Leena Vainionpää Ilmo E Hassinen Kari Majamaa

OBJECTIVES Many heteroplasmic point mutations in tRNA genes of mitochondrial DNA (mtDNA) have been associated with human diseases. We recently reported on a prospective 7-year study in which we enrolled 116 consecutive children with undefined encephalomyopathy. Seventeen of them were found to have both a defect in the mitochondrial respiratory chain and abnormal ultrastructure of muscle mitocho...

2009
Rita Horvath John P. Kemp Helen A. L. Tuppen Gavin Hudson Anders Oldfors Suely K. N. Marie Ali-Reza Moslemi Serenella Servidei Elisabeth Holme Sara Shanske Gittan Kollberg Parul Jayakar Angela Pyle Harold M. Marks Elke Holinski-Feder Mena Scavina Maggie C. Walter Jorida Çoku Andrea Günther-Scholz Paul M. Smith Robert McFarland Zofia M. A. Chrzanowska-Lightowlers Robert N. Lightowlers Michio Hirano Hanns Lochmüller Robert W. Taylor Patrick F. Chinnery Mar Tulinius Salvatore DiMauro

Childhood-onset mitochondrial encephalomyopathies are usually severe, relentlessly progressive conditions that have a fatal outcome. However, a puzzling infantile disorder, long known as 'benign cytochrome c oxidase deficiency myopathy' is an exception because it shows spontaneous recovery if infants survive the first months of life. Current investigations cannot distinguish those with a good p...

2012
Frank Jühling Joern Pütz Matthias Bernt Alexander Donath Martin Middendorf Catherine Florentz Peter F. Stadler

Transfer RNAs (tRNAs) are present in all types of cells as well as in organelles. tRNAs of animal mitochondria show a low level of primary sequence conservation and exhibit 'bizarre' secondary structures, lacking complete domains of the common cloverleaf. Such sequences are hard to detect and hence frequently missed in computational analyses and mitochondrial genome annotation. Here, we introdu...

Mitochondria contain their own DNA (mtDNA), which codes for 13 proteins (all subunits of the respiratory chain complexes), 22 tRNAs and 2 rRNAs. Several mtDNA point mutations as well as deletions have been shown to be causative in well-defined mitochondrial disorders. A mixture of mutated and wild type mtDNA (heteroplasmy) is found in most of these disorders. Inheritance of mtDNA is maternal, a...

Journal: :Fertility and sterility 1999
Z Blumenfeld B Brenner

OBJECTIVE To critically review the literature regarding inherited thrombophilia and recurrent fetal loss. DESIGN English-language literature review. PATIENT(S) Women who experienced repeated pregnancy wastage. INTERVENTION(S) Aspirin, glucocorticoids, heparin, and IV immunoglobulin for the prevention of miscarriage. MAIN OUTCOME MEASURE(S) Live birth, miscarriage, preeclampsia, and preg...

Journal: :Proceedings of the National Academy of Sciences of the United States of America 1971
A Halbreich M Rabinowitz

Formyltetrahydrofolic acid:methionyl-tRNA transformylase was isolated from Saccharomyces cerevisiae mitochondria and used to prepare yeast mitochondrial [(3)H]formylmethionyl-tRNA. This fMet-tRNA hybridizes with mitochondrial DNA but not with yeast nuclear or E. coli DNA. Unlabeled mitochondrial, but not extramitochondrial, tRNA competes in this reaction. tRNA was eluted from the hybrid and fou...

2002
F J del Castillo M Villamar M A Moreno-Pelayo J J Almela C Morera I Adiego F Moreno I del Castillo

Inherited hearing impairment is a highly heterogeneous group of disorders. 2 In a majority of cases (about 70%), the hearing loss is non-syndromic, that is, it is not associated with any other clinical feature. It can be transmitted following autosomal (recessive or dominant), X linked, or maternal inheritance patterns. In the nuclear genome, more than 70 loci have been reported to be involved ...

2015
Carla Giordano Veronica Morea Elena Perli Giulia d’Amati

Mutations in mitochondrial (mt) DNA determine important human diseases. The majority of the known pathogenic mutations are located in transfer RNA (tRNA) genes and are responsible for a wide range of currently untreatable disorders. Experimental evidence both in yeast and in human cells has shown that the detrimental effects of mt-tRNA point mutations can be attenuated by increasing the express...

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