Iron-storing disorders shed light on Parkinson's

[News <x12345@xxxxxxxxxxxxxxxx>]

                       Iron-storing disorders shed light on Parkinson's


                      July 24, 2001

                      NEW YORK (Reuters Health) - Researchers have uncovered
genetic
                      defects underlying two rare disorders in which iron
builds up in brain
                      cells, resulting in deteriorating brain function.

                      The findings may help researchers understand more
common
                      disorders, such as Parkinson's, Huntington's and
Alzheimer's disease,
                      which also involve increased levels of iron in brain
cells, researchers
                      say.

                      The two studies were reported July 23 in an online
publication of Nature
                      Genetics. In the first study, Dr. John Burn from the
Institute of Human
                      Genetics, Newcastle upon Tyne, UK, and his colleagues
identified a
                      genetic mutation that resulted in patients having
symptoms similar to
                      those of people with Huntington's or Parkinson's
disease.

                      The symptoms of the disorder, called adult-onset basal
ganglia
                      disease, include involuntary movements, spasticity and
rigidity, and
                      typically strike patients between 40 to 55 years of
age. However, the
                      patients do not show a decline in reasoning ability.

                      Burn's team examined genetic material from five
patients with the
                      disorder and compared it with that of three healthy
people. The
                      investigators found that all of the affected
individuals had a defective
                      version of an iron-storing protein, which could cause
iron to accumulate
                      in brain cells.

                      In the second paper, Dr. Susan J. Hayflick from the
Oregon Health and
                      Science University in Portland, and colleagues
describe their discovery
                      of the genetic defect underlying a rare disorder
called
                      Hallervorden-Spatz syndrome (HSS). These patients,
numbering about
                      100 in the US, also suffer severe damaging effects
from a buildup of
                      iron in their brain cells.

                      "People with Parkinson's disease have increases of
iron in their brain in
                      some of the same areas in which it is increased in
HSS," Hayflick told
                      Reuters Health. "We are speculating that some
variation in this gene
                      (that causes HSS) may be at the basis of the common
forms of
                      Parkinson's disease," she said.

                      "We are actually looking now at a group of Parkinson's
patients to see
                      if there are changes in this gene," Hayflick said. "We
don't think this
                      gene is going to be the cause of all Parkinson's
disease, but it may be
                      a significant contributor," she said.

                      According to Hayflick, the iron buildup is caused by a
mutated protein
                      that is unable to break down a B vitamin called
pantothenic acid, or
                      vitamin B5. This defect, through a chain of events,
may trigger the
                      accumulation of iron, she said.

                      Hayflick did not want to speculate on whether taking
pantothenic acid
                      as a vitamin supplement may help or hinder HSS or
Parkinson's, but
                      she said, "we are working to develop compounds (based
on
                      pantothenic acid) that would be able to get into cells
in order to treat
                      the disorder(s)."

----------------------------------------------------------------------
To sign-off Parkinsn send a message to: mailto:listserv@xxxxxxxxxxxxxxxxxxxx
In the body of the message put: signoff parkinsn