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Direct neuronal degeneration is brought on by Krabbe disease: study

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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.

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.

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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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Holi 2024: Inside, professional advice on overcoming a Bhang hangover

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Holi 2024: Inside, professional advice on overcoming a Bhang hangover

Holi 2024: We are eagerly awaiting the arrival of this festival of colors, which is quickly approaching. Every year, the nation celebrates Holi with great fanfare and extravagance. Holi commemorates the eternal love and marriage of Goddess Radha and Lord Krishna. In addition, Holi commemorates Lord Vishnu’s victory over Hiranyakashipu, highlighting the idea that good always triumphs over evil. Holi will be celebrated on March 25 this year. Holika Dahan, also known as Chhoti Holi, is observed the day before Holi. On the day of Holi, many customs and rites are observed throughout India. The cities of Barnsana and Nandgaon celebrate Lathmar Holi, whereas Vrindavan celebrates Phoolwali Holi.

Holi 2024: Here are some professional suggestions to avoid Bhang hangover, ranging from eating a balanced diet to sipping herbal tea.

During Holi, a variety of snacks and beverages are made to be enjoyed with loved ones. The colorful, crispy, and delectable Holi snacks range from gujia to rasmalai to namakpare. Thandai, a delightful concoction of milk, spices, and sweets, is the queen of the drink table. Bhang is among the primary draws of Holi. For its euphoric impact, thandai is sometimes blended with powdered paste made from the female cannabis plant’s leaves and flowers. But after the first euphoria wears off, there’s also the inevitable hangover the following morning.

Methods for overcoming a hangover

Hydrate the body: Hydration is crucial for managing a hangover caused by Bhang during Holi. Sip on lots of water to flush out toxins and replenish your body’s moisture.

Consume coconut water: Coconut water and other electrolyte-rich beverages can help replenish lost nutrients.

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A balanced diet: blood sugar regulation and nausea reduction can be achieved by eating a well-balanced meal high in carbohydrates, proteins, and healthy fats.

Herbal teas and rest: Herbal teas with relaxing properties, such as peppermint or ginger, can ease stomach discomfort. Your body can mend itself by resting in a quiet, dark environment.

Limit alcohol intake: Limit your alcohol and cannabis intake to prevent symptoms from getting worse. As soon as symptoms develop or continue, get medical help.

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