Subramaniam Ganesh


Affiliation: Indian Institute of Technology
Country: India


  1. request reprint
    Parihar R, Rai A, Ganesh S. Lafora disease: from genotype to phenotype. J Genet. 2018;97:611-624 pubmed
    ..We also discuss the disease pathologies as revealed by the studies on the animal models and, finally, on the progress with therapeutic attempts albeit in the animal models. ..
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    Ganesh S, Puri R, Singh S, Mittal S, Dubey D. Recent advances in the molecular basis of Lafora's progressive myoclonus epilepsy. J Hum Genet. 2006;51:1-8 pubmed
    ..Finally, we also discuss a possible mechanism to explain the locus heterogeneity observed in LD. ..
  3. Rai A, Singh P, Singh V, Kumar V, Mishra R, Thakur A, et al. Glycogen synthase protects neurons from cytotoxicity of mutant huntingtin by enhancing the autophagy flux. Cell Death Dis. 2018;9:201 pubmed publisher
    ..Taken together, our results thus provide a novel function for glycogen synthase in proteolytic processes and offer insight into the role of glycogen synthase and glycogen in both survival and death of the neurons. ..
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    Parihar R, Mishra R, Singh S, Jayalakshmi S, Mehndiratta M, Ganesh S. Association of the GRM4 gene variants with juvenile myoclonic epilepsy in an Indian population. J Genet. 2014;93:193-7 pubmed
  5. Upadhyay M, Gupta S, Bhadauriya P, Ganesh S. Lafora disease proteins laforin and malin negatively regulate the HIPK2-p53 cell death pathway. Biochem Biophys Res Commun. 2015;464:106-11 pubmed publisher
    ..Taken together, our study strengthens the notion that laforin and malin are pro-survival factors, and that the activation of Hipk2-p53 cell death pathway might underlie neurodegeneration in LD. ..
  6. Jain N, Mishra R, Ganesh S. FoxO3a-mediated autophagy is down-regulated in the laforin deficient mice, an animal model for Lafora progressive myoclonus epilepsy. Biochem Biophys Res Commun. 2016;474:321-327 pubmed publisher
    ..Our results suggest that FoxO3a exerts a negative control over mTOR, and its loss could result in autophagic defects in LD associated with laforin deficiency. ..
  7. Upadhyay M, Agarwal S, Bhadauriya P, Ganesh S. Loss of laforin or malin results in increased Drp1 level and concomitant mitochondrial fragmentation in Lafora disease mouse models. Neurobiol Dis. 2017;100:39-51 pubmed publisher
    ..Our results suggest that laforin and malin are novel regulators of mitochondrial quality control pathway and that the mitochondrial dysfunction resulting from the increased Drp1 levels could underlie neuropathology in LD. ..
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    Ganesh S, Tsurutani N, Suzuki T, Ueda K, Agarwala K, Osada H, et al. The Lafora disease gene product laforin interacts with HIRIP5, a phylogenetically conserved protein containing a NifU-like domain. Hum Mol Genet. 2003;12:2359-68 pubmed
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    Ganesh S, Tsurutani N, Suzuki T, Hoshii Y, Ishihara T, Delgado Escueta A, et al. The carbohydrate-binding domain of Lafora disease protein targets Lafora polyglucosan bodies. Biochem Biophys Res Commun. 2004;313:1101-9 pubmed
    ..This is the first report demonstrating a direct association between the LD gene product and the disease-defining storage product, the Lafora bodies. ..

More Information


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    Ganesh S, Tsurutani N, Amano K, Mittal S, Uchikawa C, Delgado Escueta A, et al. Transcriptional profiling of a mouse model for Lafora disease reveals dysregulation of genes involved in the expression and modification of proteins. Neurosci Lett. 2005;387:62-7 pubmed
    ..Our study may provide valuable insights into the pathophysiology of LD and may aid in developing potential therapeutic targets. ..
  2. Sengupta S, Badhwar I, Upadhyay M, Singh S, Ganesh S. Malin and laforin are essential components of a protein complex that protects cells from thermal stress. J Cell Sci. 2011;124:2277-86 pubmed publisher
    ..This study demonstrates that laforin and malin are key regulators of HSF1 and that defects in the HSF1-mediated stress response pathway might underlie some of the pathological symptoms in LD. ..
  3. Upadhyay M, Bhadauriya P, Ganesh S. Heat shock modulates the subcellular localization, stability, and activity of HIPK2. Biochem Biophys Res Commun. 2016;472:580-4 pubmed publisher
    ..Taken together our study underscores the importance of HIPK2 in stress mediated cell death, and that the HIPK2 is a generic stress kinase that gets activated by diverse set of physiological stressors. ..
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    Goenka A, Sengupta S, Pandey R, Parihar R, Mohanta G, Mukerji M, et al. Human satellite-III non-coding RNAs modulate heat-shock-induced transcriptional repression. J Cell Sci. 2016;129:3541-3552 pubmed
    ..Our results thus provide a novel insight into the regulatory role for the Sat3 transcripts in heat-shock-dependent transcriptional repression. ..
  5. Jain N, Rai A, Mishra R, Ganesh S. Loss of malin, but not laforin, results in compromised autophagic flux and proteasomal dysfunction in cells exposed to heat shock. Cell Stress Chaperones. 2017;22:307-315 pubmed publisher
    ..Loss of malin leads to reduced proteasomal activity in the heat-shocked cells. Taken together, our results suggest a distinct mode of action for laforin and malin in the heat shock-induced proteolytic processes. ..