کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
5508867 1400403 2017 8 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Mitochondrial diseases: Yeast as a model for the study of suppressors
ترجمه فارسی عنوان
بیماری های میتوکندری: مخمر به عنوان یک مدل برای مطالعه سرکوبگرها
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی زیست شیمی
چکیده انگلیسی


- Identification of six amino acids from human mt LeuRS-Cterm with suppressive effects.
- Overexpression of LINNKA rescues the defective phenotypes of several mt tRNA mutants.
- The position 311 of mt EF-Tu is involved in suppression activity.
- Rescuing activity is closely related to the amount of suppressor molecules.
- Mt EF-Tu is involved in heat stress response.

Mitochondrial (mt) tRNA gene mutations are an important cause of human morbidity and are associated with different syndromes. We have previously shown that the mitochondrial protein synthesis elongation factor EF-Tu and isolated sequences from the carboxy-terminal domain of yeast and human mt leucyl-tRNA synthetases (LeuRS), have a wide range of suppression capability among different yeast mt tRNA mutants having defective respiratory phenotype. Here we show that the rescuing capability can be restricted to a specific sequence of six amino acids from the carboxy-terminal domain of mt LeuRS. On the other hand by overexpressing a mutated version of mt EF-Tu in a yeast strain deleted for the endogenous nuclear gene we identified the specific region involved in suppression.Results support the possibility that a small peptide could correct defects associated with many mt tRNA mutations, suggesting a novel therapy for mitochondrial diseases treatment.The involvement of the mt EF-Tu in cellular heat stress response has also been suggested.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: Biochimica et Biophysica Acta (BBA) - Molecular Cell Research - Volume 1864, Issue 4, April 2017, Pages 666-673
نویسندگان
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