Indian Science Technology and Engineering facilities Map
 
Preivious Next  

  Digital Catalogue for Technology and Products Development


   Technology and Product Development

    Basic Information

Technology developed: Understanding the molecular basis of myoclonic epilepsy in Lafora disease using mice models (TP19766680310)
Category: Technology Service/Know how
Details of Inventor(s):
Inventor Institution/Organization/Company Department Designation
Prof. S. Ganesh Indian Institute of Technology (IIT) Kanpur Biological Sciences & Bioengineering
Technical Application Area: Biotechnology
If 'Other', please specify:
Please give more details of new technical application area:
Organization(s):
Indian Institute of Technology (IIT) Kanpur
Affiliated Ministry: Institution Funding (Self Supported)
Type of technology development: Indigenous
Does the technology help in replacing any import items currently
procured from outside India?
Yes
Does the technology have export potential? Yes
Category of Technology developed: Immediate Deployment
Stage of Development: Prototype Level
Please describe in detail including the TRL Level:
Ready

    Abstract:

Applications: Lafora progressive myoclonus epilepsy or Lafora disease LD is one of the five forms of inherited progressive myoclonus epilepsies in human and is caused by mutations in the gene coding for the laforin phosphatase or the malin ubiquitin ligase. Besides epilepsy, LD is characterized by the abnormally high level of glycogen and their aggregates as Lafora bodies in many tissues including the neurons. Studies on the Lafora disease mice models created by targeted deletion of the gene coding for laforin or malin have demonstrated that in addition to the epilepsy and glycogen accumulation, the animals also show impairment in autophagy a cellular process where damaged and organelles and protein aggregates are cleared using a vesicle mediated proteolytic pathway. However the physiological basis of epilepsy LD, and whether the epileptic phenotype is a cause or consequence of glycogen aggregates and or the autophagic defects have not been established. This study aims to address this very important question by using the mice models of LD laforin or malin deficient mice
Advantages: Lafora progressive myoclonus epilepsy or Lafora disease LD is one of the five forms of inherited progressive myoclonus epilepsies in human and is caused by mutations in the gene coding for the laforin phosphatase or the malin ubiquitin ligase. Besides epilepsy, LD is characterized by the abnormally high level of glycogen and their aggregates as Lafora bodies in many tissues including the neurons. Studies on the Lafora disease mice models created by targeted deletion of the gene coding for laforin or malin have demonstrated that in addition to the epilepsy and glycogen accumulation, the animals also show impairment in autophagy a cellular process where damaged and organelles and protein aggregates are cleared using a vesicle mediated proteolytic pathway. However the physiological basis of epilepsy LD, and whether the epileptic phenotype is a cause or consequence of glycogen aggregates and or the autophagic defects have not been established. This study aims to address this very important question by using the mice models of LD laforin or malin deficient mice

    Technology Inputs:

Imported Equipment/Spare Parts:
Equipment/Spare Parts Year ITC-HS Code
NA
Indigenous Equipment/Spare Parts:
Equipment/Spare Parts Year ITC-HS Code
NA
Imported Raw Materials:
Raw Materials Year ITC-HS Code
NA
Indigenous Raw Materials:
Raw Materials Year ITC-HS Code
NA
Existing R&D Facilities used:
Facilities Year ITC-HS Code
NA

   Patents & Publications:

Patents:
Filed Patents (No.) Granted Patents (No.) Year
0 0 NA
Publications:
Submitted (No.) Published (No.) Year
0 0 NA

    Commercialization Potential:

Who are the Potential Licensees?
What commercially available products address
the same problem?
Company Product Problem Addressed
Would you like to develop this invention further with
corporate research support?
Yes
Would you be interested in participating in cluster based
programs for commercialization research or business
planning for your invention?
Yes
      Submitted by: Technical Team Date of Submission: 1-5-2023



Chat Room      Write Review     Talk to Experts


THE VISION
THE MISSION
ABOUT I-STEM
It has always been the basic tenet of the Government of India, in generously funding R&D efforts at academic institutions over the years, that facilities established through such support be made available to those needing them and qualified to make use of them for their own research work
read more >>

However, this was never easy or straightforward for, among other reasons, there was no ready source of information of what facility was available and where. Thanks to the Web, it is much easier today to have a national and regional “inventory of resources”, so as to match users with the resources they need, and to do all this in an efficient and transparent manner.

This can lead to a leap in R&D productivity and greatly enhance the effectiveness of public investment. This is the motivation behind I-STEM.
read less <<
Visitor Hit Counter
Hosted at Indian Institute of Science
Copyright © 2024 I-STEM. All rights reserved.
Audited by: STQC Bengaluru.