Full Text Journal Articles by
Author Roman Lyakhovetsky

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Translation inhibition corrects aberrant localization of mutant alanine-glyoxylate aminotransferase: possible therapeutic approach for hyperoxaluria.

Ruth Belostotsky, Roman Lyakhovetsky, Michael Y Sherman, Fanny Shkedy, Shimrit Tzvi-Behr, Roi Bar, Bernd Hoppe, Björn Reusch, Bodo B Beck, Yaacov Frishberg,

Primary hyperoxaluria type 1 is a severe kidney stone disease caused by abnormalities of the peroxisomal alanine-glyoxylate aminotransferase (AGT). The most frequent mutation G170R results in aberrant mitochondrial localization of the active enzyme. To evaluate the population of peroxisome-localized AGT, we developed a quantitative Glow-AGT assay based on the self-assembly ... Read more >>

J Mol Med (Berl) (Journal of molecular medicine (Berlin, Germany))
[2018, 96(7):621-630]

Cited: 3 times

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Mutations in HAO1 encoding glycolate oxidase cause isolated glycolic aciduria.

Yaacov Frishberg, Avraham Zeharia, Roman Lyakhovetsky, Ruth Bargal, Ruth Belostotsky,

The primary hyperoxalurias are a group of recessive kidney diseases, characterised by extensive accumulation of calcium oxalate that progressively coalesces into kidney stones. Oxalate overproduction is facilitated by perturbations in the metabolism of glyoxylate, the product of glycolate oxidation, and the immediate precursor of oxalate. Glycolic aciduria associated with hyperoxaluria ... Read more >>

J Med Genet (Journal of medical genetics)
[2014, 51(8):526-529]

Cited: 15 times

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Dynamic JUNQ inclusion bodies are asymmetrically inherited in mammalian cell lines through the asymmetric partitioning of vimentin.

Mikołaj Ogrodnik, Hanna Salmonowicz, Rachel Brown, Joanna Turkowska, Władysław Średniawa, Sundararaghavan Pattabiraman, Triana Amen, Ayelet-chen Abraham, Noam Eichler, Roman Lyakhovetsky, Daniel Kaganovich,

Aging is associated with the accumulation of several types of damage: in particular, damage to the proteome. Recent work points to a conserved replicative rejuvenation mechanism that works by preventing the inheritance of damaged and misfolded proteins by specific cells during division. Asymmetric inheritance of misfolded and aggregated proteins has ... Read more >>

Proc Natl Acad Sci U S A (Proceedings of the National Academy of Sciences of the United States of America)
[2014, 111(22):8049-8054]

Cited: 57 times

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Studying lamins in invertebrate models.

Roman Lyakhovetsky, Yosef Gruenbaum,

Lamins are nuclear intermediate filament proteins that are conserved in all multicellular animals. Proteins that resemble lamins are also found in unicellular organisms and in plants. Lamins form a proteinaceous meshwork that outlines the nucleoplasmic side of the inner nuclear membrane, while a small fraction of lamin molecules is also ... Read more >>

Adv Exp Med Biol (Advances in experimental medicine and biology)
[2014, 773:245-262]

Cited: 8 times

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Compartmentalization of superoxide dismutase 1 (SOD1G93A) aggregates determines their toxicity.

Sarah J Weisberg, Roman Lyakhovetsky, Ayelet-chen Werdiger, Aaron D Gitler, Yoav Soen, Daniel Kaganovich,

Neurodegenerative diseases constitute a class of illnesses marked by pathological protein aggregation in the brains of affected individuals. Although these disorders are invariably characterized by the degeneration of highly specific subpopulations of neurons, protein aggregation occurs in all cells, which indicates that toxicity arises only in particular cell biological contexts. ... Read more >>

Proc Natl Acad Sci U S A (Proceedings of the National Academy of Sciences of the United States of America)
[2012, 109(39):15811-15816]

Cited: 57 times

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Cyclosporin-A-induced prion protein aggresomes are dynamic quality-control cellular compartments.

Tziona Ben-Gedalya, Roman Lyakhovetsky, Yifat Yedidia, Michal Bejerano-Sagie, Natalya M Kogan, Marcela Viviana Karpuj, Daniel Kaganovich, Ehud Cohen,

Despite the activity of cellular quality-control mechanisms, subsets of mature and newly synthesized polypeptides fail to fold properly and form insoluble aggregates. In some cases, protein aggregation leads to the development of human neurodegenerative maladies, including Alzheimer's and prion diseases. Aggregates of misfolded prion protein (PrP), which appear in cells ... Read more >>

J Cell Sci (Journal of cell science)
[2011, 124(Pt 11):1891-1902]

Cited: 21 times

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