The Courier & Advertiser (Fife Edition)

Worm study to aid cancer-hit family

- JAKE KEITH

ADundee family devastated by a rare form of cancer cou ld benefit from new research conducted by a local academic using worms.

Dr Anil Mehta, from Dundee University, has worked closely with the Williamson family to understand more about a gene mutation that causes aggressive­ly malignant tumours found in generation­s of the family.

Fa ther -o f - four Jo Williamson lost wife Sue, 57, just two days before Christmas in 2003 after a 10 -year f ight w ith pheochromo­cytoma, which is caused by the faulty gene.

Two of their children have inherited the faulty SDH-B gene, known as the Arg230 His mutation, and need to be regularly assessed for any dangerous tumours.

Alongside a team of researcher­s, Dr Mehta, from the university’s school of medicine, has now made some p rog ress in understand­ing how the mutation works. The academics successful­ly made a biological model of the mutation, providing a platform for investigat­ion and testing of the biological effects of the exact gene change seen in the Williamson family.

Dr M eh ta, honorary reader and honorary consultant paediatric­ian, said the y managed to “exploit” the fact that the gene has remained unchanged over millennia.

He said: “We looked for an animal that was amenable to genetic manipulati­on and whose cell structure and developmen­t from embryo to adult was fully characteri­sed.

“This led us to the worm, an elegant millimetre-sized worm called Caenorhabd­itis elegans, which has been responsibl­e for so much understand­ing in cancer biology.

“We recreated the Williamson mutation in the worm and found that this worm not only lives a shortened life span but also shows a delayed and very abnormal developmen­t characteri­stic of known pathways linked to cancer.”

They found the Williamson worm makes too much lactic acid and when this is blocked with drugs, the worm can be killed.

Dr Mehta added: “We have the possibilit­y of screening these mutant worms with drug cocktails, looking for drug candidates that might kill the worms and in turn find drugs that might also kill the cancers found in the family.”

The tumours, named pheochromo­cytoma and paragangli­oma, arise in nerve cells which are often concerned with the control of hormones related to the flight or fight response of the adrenal glands.

In 2018, the Williamson family set up the Phaeo and Para Cancer Charity to raise funds in the aim of supporting further research on the faulty gene.

The charity is working on tumours removed from affected members of the family to look for overlappin­g abnormalit­ies between the cancer genes in the tumours and the findings from the Williamson worm genes.

This work is being done with a local company called Micro Matrices and is being led by Dr Simon Plummer.

The research was undertaken by a consortium of scientists from Hungary, Singapore and India.

The findings are published online on The Company of Biologists site.

We have the possibilit­y of screening these mutant worms

 ??  ?? GENE FAULT: Rare cancer pheochromo­cytoma has taken a devastatin­g toll on the Williamson family from Dundee.
GENE FAULT: Rare cancer pheochromo­cytoma has taken a devastatin­g toll on the Williamson family from Dundee.

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