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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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Gurucharan Singh of Taarak Mehta Ka Ooltah Chashmah gets hospitalised and releases a video: “Zaada kharab ho gayi hai haalat bohot.” “Troubling News: Gurucharan Singh of Taarak Mehta Ka Ooltah Chashmah Hospitalized, Shares Worrying Update: ‘Zaada Kharab Ho Gayii Hai Haalat Bhoot'”

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2 weeks ago

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January 8, 2025

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Gurucharan Singh of Taarak Mehta Ka Ooltah Chashmah gets hospitalised and releases a video: “Zaada kharab ho gayi hai haalat bohot.” “Troubling News: Gurucharan Singh of Taarak Mehta Ka Ooltah Chashmah Hospitalized, Shares Worrying Update: ‘Zaada Kharab Ho Gayii Hai Haalat Bhoot'”

Gurucharan Singh gave a glimpse of his hospital room and himself. In the clip, he didn’t reveal the reason for getting hospitalised.
Actor Gurucharan Singh, who became famous for playing Roshan Sodhi in Taarak Mehta Ka Ooltah Chashmah, has been hospitalised. Taking to Instagram on Tuesday, Gurucharan shared a video of himself as he rested on the hospital bed.

Taarak Mehta’s Gurucharan Singh hospitalised

In the video, Gurucharan said that he was very unwell. The actor also gave a glimpse of his hospital room. He panned the camera towards his hand on which a cannula was seen. The actor was seen wearing a yellow and blue shirt. He wrapped himself in a shawl.

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What Gurucharan said about his health

The actor started the video by saying, “Haalat bohot zaada kharab ho gayi hai (My condition has gotten worse).” He then wished his fans for Gurpurab. Gurucharan continued, “My blood tests have been done. I will update you about my health soon. Haalat dekho (See my condition).” He, however, didn’t reveal the reason for getting hospitalised.

Sharing the caption, Gurucharan wrote, “DHAN DHAN SAHEB SIRI GURU GOBIND SINGH SAHEB MAHARAJ JI DE GURPURAB DIYA LAKH LAKH CRORE CRORE WADHAIYA Ji (folded hands emojis).

Kal GURPURAB te GURU SAHEB Ji ne mainu nava jeewan Bakshia, GURU SAHEB Ji nu Unlimited Infinite times Dhanvaad (Yesterday on Guru Purab, Guru Sahebji gave me a new lease on life. I thank him infinite times).”

“Ji te App saaria nu jinna de GURU SAHEB ji di Kirpa sadke Ajj aap Ji de saamne zinda haan, sabnu dilo namahkaar te Dhanvaad. Thank you to Everyone. RAB RAKHA G, TE, WAHEGURU G MEHAR KARAN G, WAHEGURU JI KA KHALSA WAHEGURU JI KI FATEH,” he concluded.

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Last year Gurucharan disappeared for weeks

Gurucharan made headlines in 2024 after he went missing in April. He returned to his Delhi home in May and said that he was facing some personal and professional issues, which led him to go on a “spiritual journey.”

In April, Gurucharan’s distressed father filed a complaint with Delhi Police, detailing his son’s sudden disappearance since his departure for Mumbai. According to his father’s statement, Gurucharan, who was visiting his parents in Delhi, was expected to return to Mumbai. But he never reached the city.

During the initial investigation, the police also found that the actor was operating multiple bank accounts for financial transactions and was frequently using credit cards despite not being in a sound fiscal state.

Gurcharan in TMKOC

Gurcharan played Roshan in Taarak Mehta Ka Ooltah Chashmah until 2012, but returned in 2013 due to his fans’ demand. He finally left the show in 2020. Balwinder Singh Suri replaced him. In a heartfelt video, Gurucharan appeared visibly weak and expressed his concerns, saying, “Zaada kharab ho gayi hai haalat bohot,” meaning his condition had deteriorated significantly.

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Gurucharan Singh, best known for his role as Sodhi in the popular TV show Taarak Mehta Ka Ooltah Chashmah, recently shared troubling news with his fans. The actor, who has been a fan favorite for years, was hospitalized due to health complications and took to social media to update his followers on his condition.

In the video, he assured his fans that he was receiving medical attention and thanked them for their support during this difficult time. His words resonated with his audience, who flooded social media with messages of concern and well-wishes for his speedy recovery. Gurucharan’s fans have been expressing their love for the actor, hoping to see him back on his feet soon.

#GurucharanSingh #TaarakMehtaKaOoltahChashmah #Hospitalized #HealthUpdate #ZaadaKharab #GetWellSoon #TMKOC #TVStars #SupportAndPrayers #RecoveryJourney

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