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Direct neuronal degeneration is brought on by Krabbe disease: study

Published

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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Allu Arjun

Hema Malini bursts out laughing at Baba Ramdev’s hazardous hair flip during holy dip at Maha kumbh. Watch

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3 weeks ago

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January 30, 2025

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Hema Malini bursts out laughing at Baba Ramdev’s hazardous hair flip during holy dip at Maha kumbh. Watch

Hema Malini took a holy dip on Mauni Amavasya, sharing a light moment with yoga guru Baba Ramdev during the event.

During the ongoing Maha Kumbh Mela 2025 in Prayagraj, a light-hearted moment between actress and BJP MP Hema Malini and yoga guru Baba Ramdev has garnered attention. The incident occurred during a holy dip in the Ganges River, a ritual observed by many devotees during the Kumbh.

As Baba Ramdev emerged from the sacred waters, he performed a vigorous hair flip to clear water from his long locks. This dramatic gesture, characteristic of his energetic persona, inadvertently splashed water towards Hema Malini, who was standing nearby. Caught off guard, Hema Malini burst into laughter, adding a spontaneous and joyful touch to the solemn occasion.

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The video capturing this candid interaction quickly spread across social media platforms, with many users appreciating the unscripted display of camaraderie between the two prominent figures. Comments highlighted the human side of public personalities, noting how such moments bridge the gap between celebrities and the general populace.

The Maha Kumbh Mela, held every 12 years, is a significant religious event attracting millions of pilgrims from around the world. This year’s gathering has seen participation from various dignitaries, spiritual leaders, and celebrities, all coming together to partake in the holy rituals.

Hema Malini, known for her illustrious career in Indian cinema and her role as a parliamentarian, has been actively participating in the events at the Kumbh. She recently recited Sanskrit shlokas and spoke on the significance of Sanatan Dharma during her address at the Maha Kumbh Sabha in Prayagraj.

During the ongoing Maha Kumbh Mela 2025 in Prayagraj, a light-hearted moment between actress and BJP MP Hema Malini and yoga guru Baba Ramdev has captured public attention. As both emerged from a holy dip in the Ganges River.

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Baba Ramdev performed a vigorous hair flip to clear water from his long locks. This energetic gesture inadvertently splashed water towards Hema Malini, who was standing nearby. Caught off guard, she burst into laughter, adding a spontaneous and joyful touch to the solemn occasion.

The video capturing this candid interaction quickly spread across social media platforms, with many users appreciating the unscripted display of camaraderie between the two prominent figures. Comments highlighted the human side of public personalities, noting how such moments bridge the gap between celebrities and the general populace.

The Maha Kumbh Mela, held every 12 years, is a significant religious event attracting millions of pilgrims from around the world. This year’s gathering has seen participation from various dignitaries, spiritual leaders, and celebrities, all coming together to partake in the holy rituals.

Hema Malini, known for her illustrious career in Indian cinema and her role as a parliamentarian, has been actively participating in the events at the Kumbh. She recently recited Sanskrit shlokas and spoke on the significance of Sanatan Dharma during her address at the Maha Kumbh Sabha in Prayagraj.

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Baba Ramdev, a renowned yoga guru and proponent of Ayurveda, to has been a prominent figure at the Kumbh, leading yoga sessions and engaging with devotees. His dynamic presence and enthusiasm have been well-received by attendees.

The shared moment between Hema Malini and Baba Ramdev serves as a reminder of the unity and shared joy that the Kumbh Mela fosters among its participants, transcending social and cultural boundaries.

For a glimpse into Hema Malini’s participation at the Maha Kumbh Sabha, you can watch the following video:


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Entertainment News Platforms – anyflix.in      
Construction Infrastructure and Mining News Platform – https://cimreviews.com/
General News Platform – https://ihtlive.com/
Legal and Laws News Platforms – https://legalmatters.in/
Podcast Platforms – https://anyfm.in/

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