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Kanazawa University research: How Cells Repair DNA’s Protective Barrier: A pathway to address a rare genetic disorder characterized by rapid aging in children

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KANAZAWA, Japan, Dec. 11, 2024 /PRNewswire/ — Researchers at Nano Life Science Institute (WPI-NanoLSI), Kanazawa University, have discovered how a protein called lamin A helps repair the protective barrier around a cell’s DNA. The findings reveal lamin A’s unique role and its potential for treating Hutchinson-Gilford Progeria Syndrome, a rare disorder that causes premature aging.

The nuclear envelope (NE) is a vital barrier that protects the cell’s genetic material. It is supported by the nuclear lamina (NL), a fibrous protein network composed of lamins, including lamin A (LA) and lamin C (LC). Mechanical stress or genetic abnormalities can cause ruptures in the NE, exposing the genetic material to damage. While lamin C rapidly accumulates at NE rupture sites to facilitate repair, lamin A exhibits slower and weaker localization.

This slower response poses significant challenges, especially in diseases like Hutchinson–Gilford Progeria Syndrome (HGPS). In HGPS, a mutation in the LMNA gene produces progerin, a defective variant of lamin A that remains permanently associated with the NE and disrupts repair mechanisms (Fig. 1). Because progerin’s impaired mobility reduces the reserve pool available for repair, cellular damage could be further compounded, contributing to accelerated aging symptoms in patients.

An international team of researchers led by Takeshi Shimi at the NanoLSI, Kanazawa University, aimed to solve a critical question: Why does lamin A localize more slowly to NE rupture sites compared to lamin C, and how does this difference impact nuclear stability in both normal and diseased states? Specifically, they sought to understand how lamin A’s unique tail region and the post-translational modifications, such as farnesylation, influence its localization and functionality.

Key Findings

Lamin A’s Tail Region
The researchers have identified specific sequences in lamin A’s tail region, termed “Lamin A-Characteristic Sequences” (LACS1 and LACS2) that inhibit its rapid localization to rupture sites (Fig.2).Progerin’s Impact in HGPS
Progerin’s defective structure leads to its permanent retention at the NE, reducing the nucleoplasmic pool of lamin A required for efficient NE repair. This delayed response contributes to nuclear instability and cellular aging.Therapeutic Potential
A farnesyltransferase inhibitor (FTI), lonafarnib (Zokinvy) improves progerin and lamin A mobility and increase its nucleoplasmic availability, significantly enhancing NE repair in both healthy and HGPS models (Fig.3). This drug is approved in the United States, Europe, and Japan for the treatment of patients with HGPS.

“This study bridges a critical gap in our understanding of Lamin A’s role in nuclear repair. It provides actionable insights for developing therapies targeting conditions where nuclear instability is a hallmark, such as HGPS,” say the authors.

Glossary

Nuclear Envelope (NE): A double membrane that encloses the nucleus, protecting the genetic material.Nuclear Lamina (NL): A fibrous network inside the nucleus that provides structural support and regulates gene expression.Lamin A (LA): A structural protein in the nuclear lamina critical for maintaining nuclear envelope stability.Hutchinson-Gilford Progeria Syndrome (HGPS): A genetic disorder caused by a mutation in the LMNA gene, leading to the production of progerin, a defective lamin A variant.Farnesylation: A lipid modification of proteins like Ras, crucial for their localization and function.Farnesyltransferase Inhibitors (FTIs): Drugs that block farnesylation, showing promise in restoring progerin and lamin A functionality.

Fig. 1 Delayed repair of nuclear envelope rupture in HGPS cells
https://nanolsi.kanazawa-u.ac.jp/wp/wp-content/uploads/Fig.1-1.png

Fig. 2 Schematic diagram of the difference in localization to the rupture sites between Lamin A, Lamin C, and Progerin
https://nanolsi.kanazawa-u.ac.jp/wp/wp-content/uploads/Fig2-5.png

Fig. 3 Delayed localization of lamin A to the rupture sites in HGPS cells
https://nanolsi.kanazawa-u.ac.jp/wp/wp-content/uploads/Fig3-3.png

Copyright for Fig. 1-3: © Kono, et al., 2024. Published by Oxford University Press on behalf of National Academy of Sciences.

Reference

Yohei Kono, Chan-Gi Pack, Takehiko Ichikawa, Arata Komatsubara, Stephen A Adam, Keisuke Miyazawa, Loïc Rolas, Sussan Nourshargh, Ohad Medalia, Robert D Goldman, Takeshi Fukuma, Hiroshi Kimura, Takeshi Shimi. Roles of the Lamin A-specific Tail Region in the Localization to Sites of Nuclear Envelope Rupture. Published online 21 November 2024. PNAS Nexus.

DOI: 10.1093/pnasnexus/pgae527
https://doi.org/10.1093/pnasnexus/pgae527

Author acknowledgements

We thank Biomaterials Analysis Division, Open Facility Center (OFC) of Tokyo Institute of Technology for nucleotide sequencing and sharing research equipment, especially Carl Zeiss ElyraS1/Airyscan/LSM780 is shared in MEXT Project Grant Number JPMXS0440200022. We also thank the Cellular Imaging Core Facility at the ConveRgence mEDIcine research cenTer (CREDIT), Asan Medical Center for technical support.

This work was supported by JSPS KAKENHI Grant Numbers JP20KK0158 (to T.S., Y.K., K.M. and H.K.), JP20K06617 (to T.S.), and JP18H05527 and JP21H04764 (to H.K.). This work was also supported by MEXT (World Premier International Research Center Initiative [WPI]). The generation and validation of the anti-murine progerin antibody was funded by the Wellcome Trust (098291/Z/12/Z to S.N.) and the British Heart Foundation (FS/IBSRF/22/25121 to L.R.).

Contact Information

Kimie Nishimura (Ms)
Project Planning and Outreach, NanoLSI Administration Office
Nano Life Science Institute, Kanazawa University
Email: nanolsi-office@adm.kanazawa-u.ac.jp 

About Nano Life Science Institute (WPI-NanoLSI), Kanazawa University

Understanding nanoscale mechanisms of life phenomena by exploring “uncharted nano-realms”.

Cells are the basic units of almost all life forms. We are developing nanoprobe technologies that allow direct imaging, analysis, and manipulation of the behavior and dynamics of important macromolecules in living organisms, such as proteins and nucleic acids, at the surface and interior of cells. We aim at acquiring a fundamental understanding of the various life phenomena at the nanoscale.

https://nanolsi.kanazawa-u.ac.jp/en/

About the World Premier International Research Center Initiative (WPI)

The WPI program was launched in 2007 by Japan’s Ministry of Education, Culture, Sports, Science and Technology (MEXT) to foster globally visible research centers boasting the highest standards and outstanding research environments. Numbering more than a dozen and operating at institutions throughout the country, these centers are given a high degree of autonomy, allowing them to engage in innovative modes of management and research. The program is administered by the Japan Society for the Promotion of Science (JSPS).

See the latest research news from the centers at the WPI News Portal: https://www.eurekalert.org/newsportal/WPI

Main WPI program site: www.jsps.go.jp/english/e-toplevel

About Kanazawa University

As the leading comprehensive university on the Sea of Japan coast, Kanazawa University has contributed greatly to higher education and academic research in Japan since it was founded in 1949. The University has three colleges and 17 schools offering courses in subjects that include medicine, computer engineering, and humanities.

The University is located on the coast of the Sea of Japan in Kanazawa – a city rich in history and culture. The city of Kanazawa has a highly respected intellectual profile since the time of the fiefdom (1598-1867). Kanazawa University is divided into two main campuses: Kakuma and Takaramachi for its approximately 10,200 students including 600 from overseas.

http://www.kanazawa-u.ac.jp/en/

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Florida Southern College Celebrates Ben and Missy Albritton Citrus and Horticulture Complex Greenhouse Raising and Miller Planetarium Rededication

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LAKELAND, Fla., Oct. 26, 2026 /PRNewswire/ — Florida Southern College celebrated two significant investments in science education and historic preservation on Thursday, Oct. 22, with the Ben and Missy Albritton Citrus and Horticulture Complex Greenhouse Raising, as well as the Miller Planetarium Rededication. The celebration highlighted the College’s commitment to agricultural education, scientific discovery, and preserving its distinctive Frank Lloyd Wright-designed campus.

The event welcomed Florida Senate President and Florida Southern alumnus Ben Albritton and his wife, Missy Albritton, along with state and local officials, College leadership, alumni, and community partners. The program included the unveiling of a dedication inscription honoring the Albritton’s, a greenhouse raising ceremony for the new greenhouse complex, and a ribbon-cutting ceremony for the newly modernized Miller Planetarium.

The Albritton Citrus and Horticulture Complex will strengthen Florida Southern’s longstanding connection to Florida’s citrus industry and agricultural education. As a student, Ben Albritton studied horticulture at the College, where faculty members helped him recognize his potential as both a student and a leader. The complex honors the Albrittons’ connection to Florida Southern and their commitment to education, agriculture, and public service.

During the greenhouse raising, invited guests signed a steel beam that will become a permanent part of the new complex. The ceremony recognized the collaborative efforts of the College, state leaders, and community supporters in advancing agricultural education. Once completed, the four new greenhouses will provide opportunities for hands-on learning, experimentation, and scientific inquiry.

State funding is also supporting improvements to Polk Science, including structural upgrades, historic preservation, and restoration of its greenhouse. Complementing these investments, a private donor gift will support renovations to existing laboratories, strengthening facilities for scientific instruction and research.

“We are particularly grateful to the State of Florida and specifically the Polk County legislators who made these opportunities possible for students, schools, and the entire community,” said Dr. Jeremy Martin, President of Florida Southern College. “We are also honored to recognize Ben and Missy Albritton, whose longstanding relationship with Florida Southern and commitment to Florida agriculture and public service make today’s celebration especially meaningful.”

The celebration continued at the Miller Planetarium, where President Jeremy Martin led the rededication and ribbon-cutting ceremony. The planetarium’s modernization includes significant upgrades to projection and audiovisual technology, transforming the historic facility into an immersive learning environment for students, K-12 school groups, families, and visitors.

The program recognized Senator Colleen Burton and Representative Jennifer Canady for their advocacy in securing state appropriations for Florida Southern over the past several years. The restoration also received support from the State of Florida, the National Park Service’s Save America’s Treasures grant program, and a private donor. These investments helped preserve the planetarium while equipping it with modern technology to inspire new generations of learners.

“The reopening of the Planetarium fulfills a dream decades in the making,” said Dr. Keith Berend, chairman of the College’s Board of Trustees. “As a student here in the late 1980s and early 1990s, I remember hoping to see this day.”

Dr. Berend also emphasized the significance of the greenhouse complex in carrying forward Florida Southern’s agricultural legacy.

“Florida Southern has a proud history in citrus education, including what was once the nation’s only bachelor’s degree dedicated to citrus. Seeing that legacy carried forward for a new generation is especially exciting,” he said.

Together, the Albritton Citrus and Horticulture Complex and the Miller Planetarium connect Florida Southern’s legacy with new opportunities for scientific exploration, student learning, and community engagement.

Founded in 1883, Florida Southern College is the oldest private college in the state. The College maintains its commitment to academic excellence through 70+ undergraduate programs and distinctive graduate programs in business administration, education, nursing, and physical therapy. Florida Southern has a 14:1 student-to-faculty ratio, is an award-winning national leader in engaged learning, and boasts 30 NCAA Division II National Championships. In the U.S. News & World Report’s 2025 “Best Colleges” guide, Florida Southern ranks #11 among “Best Regional Universities in the South,” #12 in “Most Innovative Schools,” and #24 in “Best Value Schools.” The College is also highlighted in The Princeton Review’s 2026 Best 391 Colleges guide and the “Fiske Guide to Colleges 2025.” The 2025-2026 Colleges of Distinction guidebook praises Florida Southern’s AACSB-accredited Barney Barnett School of Business and Free Enterprise alongside the College’s School of Education and its Ann Blanton Edwards School of Nursing and Health Sciences. Home to the world’s largest single-site collection of Frank Lloyd Wright architecture, Florida Southern houses 13 Wright-designed structures.

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SOURCE Florida Southern College

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The Island of The Stars, La Palma, Hosts Artemis II Commander Reid Wiseman

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CANARY ISLANDS, Spain, Oct. 26, 2026 /PRNewswire/ — From the iconic sound of Sir Brian May’s legendary Red Special guitar and the extraordinary voices of Andrea Bocelli and Sarah Brightman to the groundbreaking discoveries of Nobel Prize-winning scientists, from NASA’s Artemis II astronauts preparing for humanity’s return to the Moon to historic tributes to Stephen Hawking and Jane Goodall, STARMUS VIII — The Search for Truth brought together an extraordinary constellation of scientific minds, legendary musicians, explorers and visionary thinkers for six unforgettable days in the Canary Islands.

Held across Tenerife and La Palma from 17 to 22 October 2026, the eighth edition of STARMUS transformed the islands into a global stage for scientific discovery, artistic excellence and some of the most important conversations shaping humanity’s future.

Founded by astrophysicist Dr. Garik Israelian and legendary Queen guitarist and astrophysicist Sir Brian May, STARMUS once again demonstrated its unique ability to bridge science, music and society, bringing complex scientific ideas to audiences through extraordinary encounters, performances and shared experiences.

In an extraordinary exchange in La Palma with Reid Wiseman, Commander of the Artemis II mission to the moon, astrophysicist and Starmus co-founder Garik Israelian asked him how it felt to be at the Starmus VIII Festival in La Palma when “15 years ago we were here with Neil Armstrong Alexei Leonov, Jim Lovell, Charlie Hewitt, Buzz Aldrin, lots of scientists, Brian May….” Commander Wiseman responded, “I am here in La Palma and Alexei Leonov was one of the first people to talk to me before I launched to the International Space Station in 2014, and then Jim Lovell sent us a greeting when we went around the far side of the moon in 2014 on Artemis II…amazing to be connected through all those years.”

Media Contacts:

US: Dan DuPont, dupont0708@gmail.com, +1 202 380 7558

UK/EU: Nicole Ettinger, nicole@ettingerpr.com, +44 7515 394107

Spain: Carlos Esteban, cesteban@llys.global, +34 699 55 2854

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SOURCE Starmus Foundation

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COLUMBIA DISTRIBUTING COMPLETES ALASKA TRANSACTION WITH RNDC, ESTABLISHING A FOUR-STATE PACIFIC NORTHWEST TOTAL BEVERAGE PLATFORM

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WILSONVILLE, Ore., Oct. 26, 2026 /PRNewswire/ — Columbia Distributing today announced it has completed its transaction with Republic National Distributing Company (RNDC) to acquire certain beverage distribution assets in Alaska, marking Columbia’s official entry into the state and completing the final component of the letter of intent announced by the companies in April.

The closing represents a major milestone in a long-term strategy Columbia set out to build: a multi-category, multi-state total beverage platform across the Pacific Northwest. With direct operations now spanning Alaska, Oregon, and Washington, along with its recently completed strategic partnership with Hayden Beverage in Idaho, Columbia has brought that vision to life, creating a connected four-state route to market and a more complete one-stop solution for supplier and retail partners across the region.

“This is a huge moment for Columbia because it represents exactly what we set out to build,” said Chris Steffanci, President and CEO of Columbia Distributing. “Our long-term vision has been to create the strongest total beverage platform in the Pacific Northwest—a multi-category, multi-state strategy that gives our partners a more complete solution across the region. With Alaska, that vision is now coming to life. We can offer suppliers and retailers a connected, capable, and increasingly seamless route to market across Alaska, Idaho, Oregon, and Washington.”

The Alaska transaction expands Columbia’s direct service territory to more than 835,000 square miles and adds nearly 1,500 customer accounts. It also brings Columbia into a market with an experienced local team, strong customer relationships, and an established beverage distribution network.

Columbia sees significant untapped potential in the Alaska business and plans to bring renewed investment, leadership, resources, and market focus to the state. The company will pair the Alaska team’s local knowledge and relationships with the culture and ways of working that drive Columbia’s performance in its existing markets, empowering people, moving with urgency, holding one another accountable, and maintaining a relentless focus on customers, brands, and execution.

“What has impressed me most is the energy and ambition of the Alaska team,” Steffanci said. “They know the market, they believe in the brands, and they are hungry to grow, compete, and win. They do not need us to teach them Alaska. Our responsibility is to give them the leadership, investment, tools, and support to show what they can do.”

“At Columbia, we trust our people, empower the teams closest to the market, and expect everyone to take ownership of results,” Steffanci continued. “That culture and the way we work across our company will bring new energy and momentum to Alaska. We are not entering the market to maintain the status quo—we are here to build, grow, compete, and raise the standard. This team is ready to be unleashed, and I believe the market is going to feel the difference very quickly.”

The expansion is the latest step in the deliberate strategy Columbia has pursued to build a broader total beverage business across multiple categories and markets. On June 30, the company completed its acquisition of certain RNDC wine and spirits distribution rights in Oregon and Washington. In September, Columbia completed a strategic partnership with Hayden Beverage, creating long-term alignment with Idaho’s leading locally based beverage distributor. The Alaska transaction completes the geographic footprint Columbia envisioned across the four-state Pacific Northwest market.

Together, these transactions create a platform designed around what suppliers and retailers increasingly need: one partner capable of representing multiple beverage categories across multiple states while still maintaining the local knowledge, relationships, and market-level execution required to succeed in each community.

Columbia’s expanded platform spans beer, wine, spirits, cider, non-alcoholic beverages, and emerging beverage categories. For supplier partners, it provides greater regional scale, category expertise, operational capabilities, and a more cohesive route to market. For retailers, it provides access to a broader portfolio backed by strong sales, delivery, merchandising, revenue management, and account execution capabilities.

“This is what total beverage means to us,” Steffanci added. “It is not simply adding categories or adding geography. It is about building a one-stop solution that connects categories, capabilities, people, and markets in a way that creates more value for our partners. We now have the platform we envisioned across the Pacific Northwest, and our focus turns to executing against that opportunity.”

Columbia is working closely with the Alaska team, suppliers, customers, and business partners to support a smooth transition. Columbia will communicate additional information about the operational transition, portfolio, and go-live timing directly to affected stakeholders.

Columbia appreciates RNDC’s partnership and the work of teams across both organizations to complete the transaction. Financial terms were not disclosed.

About Columbia Distributing

Since 1935, Columbia Distributing has helped build some of the best-known brands in the beverage business. Today, Columbia is one of the nation’s leading total beverage distributors, with a portfolio spanning beer, wine, spirits, cider, non-alcoholic beverages, and emerging beverage categories.

Columbia’s more than 3,200 teammates serve approximately 24,000 retail customers and deliver more than 80 million cases annually. With direct operations across Alaska, Oregon, and Washington and a strategic partnership with Hayden Beverage in Idaho, Columbia offers supplier and retail partners a connected four-state, multi-category route to market across the Pacific Northwest.

Columbia’s success is built on its people, its brands, and a deep-rooted commitment to execution, service, and creating sustainable growth for its teammates, customers, suppliers, shareholders, and communities. For more information, visit www.coldist.com.

Contact: Steve Gibbs
Steve.Gibbs@coldist.com | 206-920-6799

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SOURCE Columbia Distributing

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