New type of CMTX - CMTX5 Discovery - males only affected

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Scientists Discover Nerve-Damaging Disease

from Hankooki.com - The Korea Times - Technology 6/14/05

By Kim Tae-gyu, Staff Reporter

A team of Korean scientists has discovered a hereditary disorder that

causes deafness, blindness and damage to peripheral nerves.

The team, led by Sungkyunkwan University professor Kim Jong-won, said

yesterday that it discovered the disease and named it CMTX5.

" It is the first time in about three decades that Korean researchers

have discovered a disease and gained global recognition since the

Hantaan virus, " Kim said.

In 1976, Lee Ho-wang, former president of the National Academy of

Science, found the cause of Korean haemorrhagic fever and called it

the Hantaan virus, which is carried by rodents.

CMTX5 patients, who are typically born deaf and lose their eyesight

later, suffer from a progressive motor and sensory neuropathy that

damages peripheral nerves during childhood.

As peripheral nerves, which relay information from the central

nervous system to other organs, become weakened, CMTX5 sufferers are

unable to walk properly and foot deformities also occur.

Kim's team found that the disorder, which occurs in less than 10

people out of 100,000, is caused by a mutation in a gene on the X

chromosome and is only diagnosed in males.

Although it severely damages peripheral nerves, CMTX5 does not affect

the central nervous system and Kim believes that this might provide

insight on understanding a range of neuropathies.

" CMTX5 proves that there exist genes in our body that control only

peripheral nerves, not affecting the central nervous system. By

pinpointing the genes, we will be able to deal with various

peripheral nerve diseases, " Kim said.

The discovery of the new pathology was featured in the latest edition

of Neurology journal.