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A Ray of Hope. A Cure for HIBM

HIBM was first described in Iran’s Jewish population in the early 1980s.
[additional-authors]
August 30, 2023
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The Jews of Iran have been living in that land for nearly 2700 years. During this time, they have survived the challenges of life as a minority, first in a predominantly Zoroastrian land, and then in a Muslim world that since c. 650 C.E. consistently regarded its Jewish citizens as dhimmis: Second-class citizens with few societal privileges, economic opportunities and legal rights. Of the numerous social challenges that have impacted the lives of Iranian Jews as dhimmis under Islamic rulers, perhaps one of the most painful has been an unintended consequence of these socio-economic restrictions that invariably forced Jews throughout Iran to remain within the community into which they were born. 

With little means for social, geographical, and/or economic mobility, the Jews of Iran have married gentile members of their respective communities for centuries. One unintentional benefit of this dynamic was that many of these Jewish communities maintained their own particular Judeo-Persian dialect — each closely rooted in various ancient Persian languages — which have preserved numerous native linguistic features that might otherwise have been lost to history. On the other hand, the bitter reality of second-class citizenship for the Jews of Iran has also brought heartbreaking repercussions — reverberations that, over the past four decades, the community has only begun to fully understand. Today, one of those repercussions is known to geneticists as GNE Myopathy (GNEM) or Hereditary Inclusion Body Myopathy (HIBM).

There has been a relatively high prevalence of HIBM among the Jews of Iran…HIBM is a rare genetic disorder that affects muscle strength and function throughout an individual’s body, leaving the patient wheelchair-bound within 10 to 15 years after the onset of symptoms. 

Hereditary Inclusion Body Myopathy is a rare genetic disorder that affects muscle strength and function throughout an individual’s body, leaving the patient wheelchair-bound within 10 to 15 years after the onset of symptoms. The disease usually affects those in early adulthood, starting in the legs and eventually leading to the complete loss of mobility. While recent studies have shown the disorder also to exist throughout the Middle East, India, Japan and some Western countries, there has been a relatively high prevalence of HIBM among the Jews of Iran, especially among the Jewish communities of Isfahan in central Iran, Kashan located between Isfahan and Tehran, and Mashhad in the northeast. Sadly, HIBM has also been detected in other Jewish Iranian communities, too. 

While rare, an estimated 10 to 15% of the population in these communities are known to carry at least a single copy of the mutated gene which codes for an enzyme known as Glucosamine (UDP-N-Acetyl)-2-Epimerase/N-Acetylmannosamine Kinase, typically abbreviated as GNE. Given the numerous restrictions on geographical mobility for the Jews of Iran, community-specific intermarriage most likely is the culprit behind the high prevalence of this genetic disorder among these specific communities.

HIBM was first described in Iran’s Jewish population in the early 1980s. Since then, researchers have identified specific genetic mutations that are responsible for causing HIBM. The disease is inherited in an autosomal recessive manner, meaning that an individual must inherit two copies of the mutated GNE gene (one from each parent) to develop the condition. If only one parent carries a copy of the mutated gene, they remain asymptomatic and there’s a 50% chance that one offspring will be an asymptomatic carrier. If both parents carry the gene, the likelihood of passing the mutation to their children is 75%. In this scenario, there’s a 25% chance that one offspring will come down with HIBM. 

Since the early days of this discovery, a community-wide effort has been made to test young couples for the gene before they’re married. Given the absence of a known cure, this solution has thus far proven the most effective means of putting an end to this potentially devastating condition. Today, unfortunately, young adults continue to find themselves facing the challenges of living with HIBM. For this reason, many efforts have been made to study the genetics of HIBM in hopes of developing potential treatments. Nevertheless, HIBM has remained a challenging condition with no known cure. But today, there is hope. 

Recent advances in a wide range of genetic therapies have now brought some hope to those living with HIBM. This is a result of the work of Solve GNE, a Los Angeles-based nonprofit organization that has raised over $2.5 million and sponsored research with multiple leading medical researchers to help develop a cure for HIBM. The key to this organization lies in its collaboration within the research community. 

Since its inception in 2022, Solve GNE has supported a consortium of academic and private sector researchers tasked with the development of various gene therapies. To this end, Solve GNE has agreed to fund the work of four leading organizations: Genosera, Gradalis, Johns Hopkins University School of Medicine and Stanford University. These agreements include commitments from Genosera and Gradalis to start human clinical trials within 12 to 18 months. 

Solve GNE is focusing on a common goal; using this a parallel track strategy should allow them to approach the FDA with multiple options to start Phase 1 trials as rapidly as possible. 

Each participant brings a unique perspective to the collaboration. Solve GNE will allocate funding accordingly to the four institutions. Additionally, Solve GNE has identified Nationwide Children’s Hospital in Ohio and Stanford University as primary clinical sites.

Solve GNE will host a global webcast reporting their progress on Sunday, Sept. 10 at 8:00 AM PDT. For more information and a link to the webcast, please visit www.solvegne.org or follow them on Facebook and Instagram.


Houman M. Sarshar is an independent scholar with numerous publications about the history of Jews in Iran, including “Esther’s Children: A Portrait of Iranian Jews” (editor) and “Jewish Communities of Iran: Entries on the Judeo-Persian Communities of Iran” (editor), published by the Encyclopædia Iranica He is also a contributing author to the Encyclopædia Iranica and the Encyclopedia of Jews in the Middle East.

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