A Hebrew University of Jerusalem study that found
why Alzheimer's develops more rapidly among people who carry a specific
mutated gene - a gene which appears in a fifth of Americans and
Israelis - is arousing much interest among dementia scientists, as it
offers a promising approach to help treat the disease.
Silberman Institute of Life Sciences finally solved a mystery
about the BChE-K gene and published their findings in the Journal of Biological Chemistry
, which featured it on the cover and selected it as its "Paper of the Week."
In theory, the carriers of the mutated gene should actually be
more protected from the devastating effects of the disease than those
with a normal gene because the altered protein the normal one produces
breaks down the neurotransmitter acetylcholine at a slower rate than in
those who have the normal version.The result is that the carriers
maintain higher levels of this neurotransmitter, so they should
theoretically be protected from Alzheimer's, in which acetylcholine
Indeed, these carriers tend to develop the disease later than others, but when it does
it progresses more rapidly and does not respond to medication. The
bottom line is that carriers of the mutated gene have a greater risk
for disease progression. The reason for this anomalous situation has
been a puzzle for a long time, but the Hebrew University scientists
solved it, thereby offering as well a possible new therapeutic
The Jerusalem researchers found that the mutation in
the BChE-K gene damages the very end - or tail - of the resultant
mutant enzyme protein. This tail is the part of BChE that protects
against Alzheimer's amyloid plaques in the brain. It does this by
interacting with the disease's b-amyloid protein and preventing it from
precipitating and forming those brain plaques that are the
neuropathological hallmark of this disease. To compare the normal
protein to the K mutant, the researchers used synthetic tails of the
normal and the K proteins, as well as engineered human BChE produced in
the milk of transgenic goats at a US company, Pharmathene. The
goat-produced protein is prepared at Pharmathene for the US military as
protection from nerve gas poisoning (a result of earlier research at
HU). It was much more stable and efficient than the mutant protein,
which suggests that the BChE-K carriers' susceptibility to Alzheimer's
could be substantially improved by treating them with the engineered
normal protein produced in the milk of the transgenic goats.
The current study was the last part in the Ph.D. work of Dr.
Erez Podoly, now working as a post-doctoral fellow with Nobel
Prof. Roger Kornberg
at Stanford University
. Podoly was the joint
student of Prof Oded Livnah and Prof. Hermona Soreq and won a National
Eshkol fellowship in biotechnology to perform this work as well as a
Kaye Innovation Award
at the Hebrew University.
The project is patented and is available for licensing by HU's Yissum Research Development Company.
PREPARING FOR EMERGENCIES
Terror attacks, missiles, catastrophic accidents in the
chemicals industry and natural disasters are all emergencies that
threaten the population every day. A graduate study program at the
University of Haifa's department of geography and environmental studies
will train its graduates to cope with such threats - from formulating
planning procedures and preventative measures to coping with events
after they occur and providing for appropriate rehabilitation.
"Many countries around the world are already placing an
emphasis on priming for disaster. Imparting professional knowledge for
those who will be decision makers and who will be required to act in
real time will make Israel
better able to manage such events," says Dr.
Sigal Blumenfeld, who is coordinating the program.
The new program will provide a comprehensive view of the field
with the help of leading lecturers. It will focus on providing
applicable tools for decision makers, including hazard-evaluation
workshops, and participation in a disaster-management forum. "The
program will deal with day-to-day coping skills in the case of an
emergency, from appropriate planning, use of technology that can
prevent particular disasters and the use of internal and external
regulation tools. Correct action in these areas will make interception,
support and rehabilitation much easier and more effective," Blumenfeld
adds. The expected students include policy makers and bureaucrats in
the public and private sectors such as emergency economy, home front,
police, fire brigade and industry security.
NHS LEARNS FROM TEREM
Twelve representatives of the British National Health Service
(NHS) came recently to Jerusalem to study TEREM, the private urgent
medical care service, and implement some of its techniques and
services. The physicians and medical administrators were fascinated by
TEREM's advanced digital technologies that make it possible for clinic
doctors to consult in real time with colleagues who are not there. Data
can be sent to a patient's family physician's computer or cellular
phone. Called Parpar, the software was a finalist in a international
Microsoft medical software competition, and will be adopted for use in
emergency rooms throughout the UK. Patients will be sent home within
two hours of their arrival, as in TEREM clinics, thanks to the
FOUR FINGERS TOO MANY
A two-year-old boy suffering from a rare genetic disorder that
gave him seven fingers on each hand has undergone three hours of
surgery at Kaplan Medical Center to make him look like any other child.
The successful operation carried out in the hand surgery department of
hospital required general anesthesia. The genetic defect
has existed in the family for six generations, but no member has had as
many as seven fingers on each hand. "We saw in the ultrasound scan
before his birth that he had 14 fingers, and this was a record in the
family," said the boy's father.
The complicated surgery required preserving the
necessary soft tissue and the flexibility of the joints so they could
function properly as the child grows, as well as attention to
esthetics, said Dr. Abraham Hess, the department's director. The
mother, who said she was much relieved as her son will be able to be
like any of his peers, is pregnant. "If and when we need surgery again,
I will not hesitate to return to Kaplan," she said.
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