CSPCA Charitable Trust
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Amyloidosis
Dr. Gary Johnson Reports to CSPCA Members
Reprinted from September/October 1996 BARKER
I have been asked to summarize remarks presented at the recent
CSPCA National Specialty concerning a disease sometimes called "Swollen
Hock Syndrome" or "Shar-Pei Fever". Typically, affected Shar-Pei
experience repeated episodes of fever and/or lameness. Unfortunately,
during these inflammatory episodes, amyloid accumulates in the kidneys
and eventually destroys these organs, leading to premature death.
Shar-Pei Fever is believed to be an inherited disease because efforts
to identify an infectious agent have been unsuccessful.
My involvement in Shar-Pei Fever research began about three years
ago when I became aware of a theory by Dr. Linda Tintle and her
colleagues that Shar-Pei Fever is the canine version of a human disease
known as Mediterranean Fever. This idea seems reasonable because
symptoms of the human disease closely resemble Shar-Pei Fever.
Mediterranean Fever was known to be an inherited genetic defect and the
responsible mutation was known to reside on the short arm of human
chromosome 16. As part of an NIH-supported project, I had developed
some DNA markers for genes from corresponding regions of dog
chromosomal DNA. I, therefore, contacted Dr. Tintle to see if she could
supply me with blood samples from families of Shar-Pei so that I could
determine if these markers would be useful in identifying dogs that
would eventually develop the disease or pass the disease onto their
offspring. As the result of subsequent chromosomal maping experiments,
the position of the Mediterranean Fever mutation is now known much more
precisely than it was three years ago. As it turned out, our markers
were from the correct half of chromosome 16, but they were too far away
from the Mediterranean Fever defect to be useful, even if Dr. Tintle's
theory is correct.
A little over a year ago, Dr. Tintle informed me of an effort to
raise $50,000 to support research on Shar-Pei Fever. Because this money
could be used to develop additional canine DNA markers closer to the
Mediterranean Fever mutation, I told her I was interested and, in fact,
would be able to contribute an addition al $25,000 in discretionary
funds toward the project. More recently, we have submitted a success-
ful Shar-Pei Fever grant proposal to the Canine Health Foundation for
an additional $25,000, bringing the total funds for the project to
$100,000. These funds are now in the hands of administrators,
accountants and lawyers; however, I am confident they will become
available to me soon so that I can begin this project in earnest.
The discretionary funds mentioned above stem from a project we are
doing for the North American Limousin Foundation which promotes and
registers a breed of beef cattle. In the past, a substantial number of
these cattle produced calves with protoporphyria, an inherited severe
photosensitivity (tendency toward severe sunburn). With support from
the NALF, we developed a DNA marker that enables us to identify
symptomless carriers of the disease. By excluding carriers from their
breeding stock, cattlemen have been able to eliminate protoporphyria
from their herds. In less than three years, we have tested over 5,000
head of cattle and greatly decreased the prevalence of protoporphyria
in Limousin cattle in this country.
If Dr. Tintle's theory about the relationship of Shar-Pei Fever
and Mediterranean Fever is correct, we have a good chance of developing
a DNA marker that identifies carriers of Shar-Pei Fever. If so, a
simple, reliable and inexpensive blood test could be used to select
mutation-free Shar-Pei for breeding puppies that should live long,
healthy lives.
CSPCA Charital Trust
Amoyloidosis/By Dr. Linda J. M. Tintle, DVM
Back to Mint Creek Valley Kennel
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CSPCA Charitable Trust
Reprinted from September/October 1996 BARKER
I have been asked to summarize remarks presented at the recent
CSPCA National Specialty concerning a disease sometimes called "Swollen
Hock Syndrome" or "Shar-Pei Fever". Typically, affected Shar-Pei
experience repeated episodes of fever and/or lameness. Unfortunately,
during these inflammatory episodes, amyloid accumulates in the kidneys
and eventually destroys these organs, leading to premature death.
Shar-Pei Fever is believed to be an inherited disease because efforts
to identify an infectious agent have been unsuccessful.
My involvement in Shar-Pei Fever research began about three years
ago when I became aware of a theory by Dr. Linda Tintle and her
colleagues that Shar-Pei Fever is the canine version of a human disease
known as Mediterranean Fever. This idea seems reasonable because
symptoms of the human disease closely resemble Shar-Pei Fever.
Mediterranean Fever was known to be an inherited genetic defect and the
responsible mutation was known to reside on the short arm of human
chromosome 16. As part of an NIH-supported project, I had developed
some DNA markers for genes from corresponding regions of dog
chromosomal DNA. I, therefore, contacted Dr. Tintle to see if she could
supply me with blood samples from families of Shar-Pei so that I could
determine if these markers would be useful in identifying dogs that
would eventually develop the disease or pass the disease onto their
offspring. As the result of subsequent chromosomal maping experiments,
the position of the Mediterranean Fever mutation is now known much more
precisely than it was three years ago. As it turned out, our markers
were from the correct half of chromosome 16, but they were too far away
from the Mediterranean Fever defect to be useful, even if Dr. Tintle's
theory is correct.
A little over a year ago, Dr. Tintle informed me of an effort to
raise $50,000 to support research on Shar-Pei Fever. Because this money
could be used to develop additional canine DNA markers closer to the
Mediterranean Fever mutation, I told her I was interested and, in fact,
would be able to contribute an addition al $25,000 in discretionary
funds toward the project. More recently, we have submitted a success-
ful Shar-Pei Fever grant proposal to the Canine Health Foundation for
an additional $25,000, bringing the total funds for the project to
$100,000. These funds are now in the hands of administrators,
accountants and lawyers; however, I am confident they will become
available to me soon so that I can begin this project in earnest.
The discretionary funds mentioned above stem from a project we are
doing for the North American Limousin Foundation which promotes and
registers a breed of beef cattle. In the past, a substantial number of
these cattle produced calves with protoporphyria, an inherited severe
photosensitivity (tendency toward severe sunburn). With support from
the NALF, we developed a DNA marker that enables us to identify
symptomless carriers of the disease. By excluding carriers from their
breeding stock, cattlemen have been able to eliminate protoporphyria
from their herds. In less than three years, we have tested over 5,000
head of cattle and greatly decreased the prevalence of protoporphyria
in Limousin cattle in this country.
If Dr. Tintle's theory about the relationship of Shar-Pei Fever
and Mediterranean Fever is correct, we have a good chance of developing
a DNA marker that identifies carriers of Shar-Pei Fever. If so, a
simple, reliable and inexpensive blood test could be used to select
mutation-free Shar-Pei for breeding puppies that should live long,
healthy lives.This page hosted by
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