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Japanese government panel OKs expensive gene therapy for rare disease

Feb 27 (Japan Times) - A health ministry panel approved Wednesday the production and sale of a gene therapy for treating spinal muscular atrophy, a rare incurable disease that destroys muscle function through a decrease in motor neuron cells.

The health minister is expected to formally approve the drug, Zolgensma, by the end of March, with public health insurance coverage seen starting this summer.

The one-time-only gene therapy, developed by Swiss drug giant Novartis AG, will cover patients below 2 years old with abnormalities in their genes to make proteins necessary for maintaining motor nerves. Novartis estimates 15 to 20 children a year are likely to receive treatment using the drug in Japan.

In the therapy, proteins are created after the genes, carried by adeno-associated viruses, are delivered to motor nerves through intravenous drips. In an overseas clinical trial, all of the 15 severe SMA patients who used the therapy were able to live without an artificial ventilator. If no treatment is offered, about 75 percent of such patients are expected to die or need an artificial ventilator before they reach the age of 13.6 months.

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Bear sightings across Japan have already climbed to nearly twice the level recorded during the same period last year, prompting entry bans in mountain areas behind Kyoto’s Ninna-ji Temple and the cancellation of hiking events in Kansai, while new research suggests that the key to reducing encounters may lie in understanding what bears eat in each region.

Copper roofing panels were stolen from several shrines in Hamamatsu, Shizuoka Prefecture, including a city-designated cultural property, in the latest case amid a nationwide surge in copper thefts targeting shrines and temples across Japan, where soaring metal prices have fueled crimes that leave historic religious buildings damaged, exposed to the elements, and facing repair costs of millions of yen.

Flames broke out on the morning of May 20th on Miyajima Island in Hiroshima Prefecture, home to one of Japan's World Heritage sites, destroying Reikado Hall near the summit of Mount Misen.

Uncertainty surrounding the situation in the Middle East is beginning to affect daily life in Japan, as concerns over crude oil supplies spread to restaurants, cleaning services and even household garbage disposal systems across the Kansai region.

A 25-year-old woman arrested as a suspected ringleader in a robbery-murder case in Tochigi Prefecture once posted cheerful dance videos on social media and was remembered by those who knew her as an energetic and outgoing young woman.

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A fire that broke out in Kagamino, Okayama Prefecture, shortly after noon on May 20th destroyed three buildings, including a home, after flames from open burning spread to dead leaves and then to nearby structures.

Six people, including a senior member of a group affiliated with the Sumiyoshi-kai crime syndicate's Kohei-ikka faction, have been arrested on suspicion of opening a gang office in a prohibited area near a nursery school in Tokyo's Itabashi Ward.

A man who visited a police station in Hiratsuka, Kanagawa Prefecture, in the early hours of May 21st allegedly sprayed a transparent liquid inside the building, causing six police officers to complain of eye and throat pain and be taken to hospital with minor injuries.

The Tokyo Metropolitan Police Department held a review ceremony for its riot police units at Meiji Jingu Gaien in Tokyo on May 20th, with around 1,700 officers marching in formation as part of a large-scale demonstration of security preparedness.

A 25-year-old woman arrested as a suspected ringleader in a robbery-murder case in Tochigi Prefecture once posted cheerful dance videos on social media and was remembered by those who knew her as an energetic and outgoing young woman.

Two women were found dead with stab wounds at a house in Tatsuno, Hyogo Prefecture, on May 19th, with police suspecting they were victims of a violent crime.

Bear attacks continue to occur across Japan, while a new problem has emerged as false reports of bear sightings flood local alert systems, placing growing pressure on municipal authorities and emergency responders.

A man in his 30s was referred to prosecutors after allegedly feeding a chocolate snack to a marmot at an animal cafe in Osaka Prefecture, despite the risk that the treat could cause poisoning or even death in the squirrel-family animal.