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Direct neuronal degeneration is brought on by Krabbe disease: study

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3 years ago

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Direct neuronal degeneration is brought on by Krabbe disease: study

A recent study found that the gene mutation underlying Krabbe disease directly causes neuronal degeneration, independent of how it affects other cell types. This new mode of action for the mutated gene provides a clearer view of the illness process and could aid in the creation of new treatments.

Galactosylceramidase (GALC) gene mutations are the primary cause of the uncommon autosomal recessive neurological ailment known as Krabbe disease. The lysosomes contain the GALC enzyme, and its lack causes the lipid psychosine to accumulate. Cell membrane instability, degeneration, and cell death are brought on by the buildup of psychosine in the brain and other areas of the body.

One of the main pathological characteristics of Krabbe disease is the loss of myelin insulation around the nerves. Oligodendrocytes, which produce myelin, have naturally been considered to be the disease’s initiators, with the degeneration of neurons acting as a later effect of this myelin loss.

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Other, more recent research has suggested that neurons may be impacted independently, but it has been challenging to test that theory because GALC is widely expressed in the brain and its loss due to disease affects all cell types. To get around this obstacle, the authors developed a mouse model in which GALC expression was only suppressed in neurons while the regular gene was still active elsewhere.

Researchers discovered that psychosine built up in neurons, causing irregularly shaped lysosomes, enlarged axons, and an increase in neuronal mortality, as well as neuroinflammation and deficiencies in mice’s motor function and coordination. No oligodendrocytes were lost, but the absence of neuronal GALC expression did result in a decrease in myelination, perhaps as a result of toxic effects on myelin sheaths from the accumulating psychosine.

For the first time, according to Shin’s findings, galactosylceramidase production in neurons is necessary to preserve and safeguard neuronal function, regardless of how it may affect oligodendrocytes that produce myelin. These findings imply that the lack of the enzyme in neurons may directly contribute to the pathogenesis of Krabbe disease, and that Krabbe disease treatments may require to target the lack of galactosylceramidase production in neurons.

Adding, Shin “Our research is the first effort to specifically examine the neuronal function of the Krabbe disease gene galactosylceramidase in a preclinical live animal model. We discovered an intrinsic neuronal role for this enzyme in creating a neuron-specific mutant of Krabbe disease. This finding suggests that, independent of myelin and other brain cell types, neuronal galactosylceramidase has a primary role in maintaining neuronal homeostasis, and that galactosylceramidase-depleted neurons may be the main cause of Krabbe disease. Galactosylceramidase supplementation to neurons would probably increase the effectiveness of therapeutic approaches for Krabbe illness since the protective role of neuronal galactosylceramidase suggests a new function unrelated to its conventional role in myelination.”

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Maharashtra HSC Result 2025: MSBSHSE Class 12th results declared at mahahsscboard.in, direct link here

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2 days ago

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May 6, 2025

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Maharashtra HSC Result 2025: MSBSHSE Class 12th results declared at mahahsscboard.in, direct link here

The Maharashtra HSC 2025 results have been released. Here is the direct link to view the MSBSHSE Class 12th results. The Maharashtra HSC Result 2025 has been released by the Maharashtra State Board of Secondary and Higher Secondary Education. The general course results for the Science, Arts, and Commerce streams of the Maharashtra HSC have released.

Students who appeared can also view their HSC results on the Daglocked result page at together results.digilocker.gov.in in addition to the official websites By following the instructions below, candidates who took the Class 12 board test can view their results. Go to mahahsscboard.in, the MSBSHSE’s official website Select the link for Maharashtra HSC Result.

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2025 that is on the main page Candidates must input their login information on a new page that opens The Maharashtra State Board of Secondary and Higher Secondary Education TO (MSBSHSE) has officially declared the Class 12 (HSC) results for 2025. Students who appeared for the exams can now access their results online.

Maharashtra HSC Result 2025: Key Details

How to Check Your Maharashtra HSC Result 2025

  1. Visit the Official Website: Go to mahresult.nic.in.
  2. Click on the Result Link: Find and click on the “Maharashtra HSC TO Result 2025” link Class 12 board test can view their results.
  3. Enter Required Details: Input your roll number and your mother’s first name. If the mother’s name Class 12 board test can view their results was not provided during registration, enter “XXX”.
  4. Submit Information: Click on the “View Result” button.
  5. View and Download: Your result will be displayed on the screen. Download and save it for future reference.Shiksha+7Mahatma Gandhi Medical College+7Best Schools Explorer+7Shiksha+4Best Schools Explorer+4Mahatma Gandhi Medical College+4

📱 Alternative Methods to Access Results

Stream-wise Pass Percentage (2024 for Reference)

Next Steps After Checking Results

  • Collect Original Marksheet: The online result is provisional. Collect the official marksheet from your school.
  • Apply for Re-evaluation: If unsatisfied with your marks, you can apply for re-evaluation through the official website.
  • Supplementary Exams: Students who did not pass can register for supplementary exams, typically held in July-August.
  • Plan for Higher Education: Based on your results, explore undergraduate programs or professional courses that align with your career goals For more detailed information and updates, visit the official MSBSHSE website. The Maharashtra State Board of Secondary and Higher Secondary Education.
  • (MSBSHSE) has declared the Class 12 (HSC) results for 2025 today, May 5. Students can access their results on official websites such as and view the results, students need to enter their roll number and mother’s first name.
  • This year, the overall pass percentage stands at 91.88%, with pass rate of 96.74%. The Science stream recorded a pass Arts 80.53% Students are advised to download and save their provisional mark sheets for future reference. The original mark sheets will be distributed by respective schools in due course.
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