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From: TSS ()
Subject: Collected tissue may be used in cosmetic surgery, Families of donors rarely get full story FAR PROFIT LifeCell $ LifeGift $ CJD/TSEs ?
Date: February 13, 2005 at 8:01 am PST

-------- Original Message --------
Subject: Collected tissue may be used in cosmetic surgery, Families of donors rarely get full story FAR PROFIT LifeCell $ LifeGift $ CJD/TSEs ?
Date: Sun, 13 Feb 2005 10:00:14 -0600
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

##################### Bovine Spongiform Encephalopathy #####################

Feb. 12, 2005, 11:14PM

Families of donors rarely get full story

Collected tissue may be used in cosmetic surgery

Copyright 2005 Houston Chronicle

Every year, hundreds of Houstonians and other Texans donate the tissues
of their deceased loved ones to help the living.

In times of deep heartbreak, families make the wrenching decision to
allow the skin, bones, heart valves and leg veins of their relatives to
be removed so that they may be transplanted into burn victims, the
disabled or diabetics.

But rarely are they told by representatives of LifeGift Organ Donation
Center — the nonprofit agency handling tissue donation cases in the
Houston area — that the tissue could potentially be used for cosmetic
surgeries. Nor are they routinely informed that it could be processed
and distributed by for-profit companies traded on Wall Street, helping
fuel a $1 billion-a-year tissue industry.

"I don't think donors' families really understand that there is a
for-profit dimension to tissue procurement," said Arthur Caplan, a
bioethicist at the University of Pennsylvania School of Medicine. "We
know that tissue companies have made very big profits and have built
lavish headquarters and are doing well."

Generally, the companies, as well as their nonprofit counterparts,
strike agreements with organizations such as LifeGift to supply them
tissue, which they then make into different products and sell to doctors
and hospitals for a wide variety of uses. Among them are skin grafts,
bone transplants, heart-valve replacements and, sometimes, cosmetic surgery.

Disclosure not required

LifeGift officials say federal law hasn't required them to tell families
— who receive no compensation — all the possible uses of donated tissue
or that they deal with for-profit companies.

That may soon change.

The federal Centers for Medicare & Medicaid Services, which regulates
organizations such as LifeGift, proposed a rule Feb. 4 that would
require companies to fully disclose all possible uses of donated tissue,
including cosmetic surgery, as well as the for-profit or nonprofit
status of companies receiving the tissues.

In anticipation of the rule, LifeGift officials are revising their
family-consent policy — and could implement it as early as this week.

"Now since the regulations require it, it is the right thing to do, and
we will do it," said LifeGift spokeswoman Catherine Burch Graham.

Carla Buchinger, who agreed to donate all of her 18-year-old son's
tissues after he died in a 2000 car accident, said the issue of cosmetic
surgery didn't arise when LifeGift approached her and asked for her
consent. Nor did it tell her his tissues may have been sent to publicly
traded companies.

Knowing that wouldn't have changed her mind, but she thinks families
should be informed.

"The fairest thing is to go ahead and tell them so that they can make an
informed decision," she said.

Advocates for donor families applaud the proposed rule.

"Our position is that families should be given as much information as
possible," said Rose D'Acquisto, who heads the volunteer committee of
the National Donor Family Council.

But she and others are concerned that some family members may say no to
donation if they learn that private companies are trying to make a
profit from the tissue of their loved ones.

"I do think we need full disclosure to families," said Penny Powers,
manager of transplant service at St. Luke's Episcopal Hospital. "But I
do worry that in such a time of stress and grieving that hearing that
there could be a for-profit element could turn them off.''

Donations tightly regulated

Each year, there are about 20,000 lifesaving heart, liver, kidney and
other organ transplants in the nation. Organ donations are tightly
controlled by the federal government, which has established a national
waiting list and offers reimbursement to 59 government-recognized Organ
Procurement Organizations across the country.

These so-called OPOs, which cover specific geographic regions, are
generally responsible for identifying potential organ donors, obtaining
consent and retrieving organs. LifeGift is the OPO for Southeast, West
and North Texas, and most of its operations are dedicated to organ
identification and recovery.

Like many OPOs, LifeGift also oversees tissue donation, which is
different. Because tissue from a donor's body can be recovered up to 24
hours after death if it is refrigerated, a far greater number of people
are potential tissue donors. And because so many tissues can be
recovered from a body, dozens of transplantations can result. Also,
there is no federal reimbursement for tissue recovery.

There are hundreds of ways donated tissue is used to improve the lives
of others.

A donor's fresh skin, for example, can be used to help burn victims, or
it can be processed to be used in medically necessary reconstructive
surgeries such as abdominal wall replacements. Bone may be crushed,
reshaped or made into powder to be used in orthopedic surgeries. Heart
valves may be transplanted into patients with heart defects. And
saphenous veins replace those of diabetics or others with such severe
circulation problems that they might be facing amputation.

About 1 million tissue transplantations are done each year, said Robert
Rigney Jr., executive director of the American Association of Tissue
Banks, which accredits about 90 tissue banks.

According to federal law, every hospital must contact its local OPO each
time a patient dies or is pronounced brain-dead to determine whether he
or she is eligible for organ or tissue donation. In most cases, organs
are not recoverable, but often tissues are.

32% agree to donate

Locally, if the deceased is an eligible donor, a LifeGift employee
approaches family members — sometimes in person and other times by phone
— and asks for their consent.

Generally, the families are told that their loved ones' tissues could go
to save or improve the lives of others, said Sean Conley, LifeGift's
manager of clinical communications and logistics. Each tissue is
described, and a general explanation of how it might be used is
reviewed, he said.

Although there is some mention of a processing and distribution system,
the families are not told — unless they ask — that some of the tissue
may be sent to for-profit companies, he said.

Last year, 32 percent of the families approached by LifeGift agreed to

The tissue is then recovered by trained, four-person teams in surgical
procedures that last up to four hours. The body is closed up carefully
enough, including the insertion of PVC piping to replace bones, so that
family members can have an open-casket funeral. And the tissues are
packaged, placed in coolers and sent to one or more of six banks that
have active contracts with LifeGift for their tissue supplies.

'Reasonable' recovery fees

The National Organ Transplant Act forbids the sale of body parts for
profit. But it does allow OPOs and banks to charge "reasonable" recovery

LifeGift officials refused to disclose its detailed recovery fees per
tissue type. But Graham said that its average total recovery fee per
donor in 2004 was about $4,800.

Former employees said the charges can be much more — about $6,000 for a
full bone recovery alone, and $10,000 or more when skin, heart valves
and veins are included.

And LifeGift officials said they recently raised their recovery rates —
between 3 percent and 15 percent — to pay for increased costs.

One of banks in Texas

One of the six banks that receive tissue from LifeGift is in Texas — the
Shriner's Burn Hospital for Children in Galveston, which uses skin to
treat burn victims.

The others are: the Musculoskeletal Transplant Foundation of Edison,
N.J., which processes bone; LifeNet of Virginia Beach, Va., which
processes heart valves; Community Tissue Services of Dayton, Ohio, which
processes bone and skin; LifeCell of Branchburg, N.J., which processes
skin; and Alabama Tissue Center of Birmingham, Ala., a subsidiary of
Regeneration Technologies, which processes heart valves and veins.

Graham said Shriner's receives all of the skin it needs from LifeGift
and that it sends only surplus to LifeCell.

LifeCell and Regeneration Technologies are for-profit companies, with
combined revenues in 2003 of $116 million, according to their annual

Each has a Web site touting its products, and they spend tens of
millions of dollars a year on marketing.

Concerns about publicity

And they worry about negative publicity. LifeCell, which makes a skin
product called AlloDerm that is used for elective cosmetic surgery,
including lip enhancements and penile enlargements, said in its 2004
annual report that it was concerned that public knowledge of this
potential use could hurt its bottom line.

"Although we do not promote the use of human tissue products for
cosmetic applications, clinicians may use our products in applications
or procedures that may be considered cosmetic," the company said.
"Negative publicity concerning the use of donated human tissue in
cosmetic procedures could reduce the demand for our products or
negatively impact the willingness of families of potential donors to
agree to donate tissue or tissue banks to provide tissue to us for

Scott Bottenfield, LifeCell's director of tissue services and partner
relations, said LifeGift "is probably our second-largest provider of skin."

But he and Graham said the vast majority of the skin — 89 percent — sent
to LifeCell in 2004 was used to make a product used almost exclusively
for burn patients, in keeping with the organization's mission to help
the sick or injured. Another 11 percent of thicker skin recovered by
"accident" was used to make AlloDerm, she and Bottenfield said.

Based on LifeCell's analysis of where the AlloDerm was sent, none of the
skin from LifeGift was likely used for elective cosmetic surgery in
2004, they said.

Still, Bottenfield and Graham said that even though it didn't happen in
2004, they can't guarantee that no skin from LifeGift will end up being
used in so-called "vanity" surgeries.

"We can't control what a surgeon is going to do, but that is not what we
are about," Bottenfield said.

Millions spent on R&D

LifeGift chief executive Sam Holtzman said the for-profit companies play
an important role because in addition to providing products that save
and enhance the lives of the sick and injured, they spend millions of
dollars a year on research and development of new products.

He said LifeGift's contracts with all of its banks — nonprofit and
for-profit alike — urge them to send as much processed tissue as
possible back to the Houston area.

But LifeCell's Bottenfield said he couldn't say for sure whether that is
happening with his products.

"We have a commitment to serve the local community," he said. "But I am
not even sure that it is likely that a lot of the skin recovered in
Houston comes back to Houston."

Holtzman acknowledged LifeGift can't always guarantee where it goes or
how it is used.

"We don't always know what the end use of a tissue product is," he said.
"Sometimes it's in storage for months or years before it can be used."

Some doctors who use those processed tissue products say families who
consent to donate should know that.

"When someone gives a gift of their loved ones tissue, they are doing it
to save a life," said Dr. Sherwin Siff, chief of orthopedic surgery at
St. Luke's and clinical professor at Baylor College of Medicine. "They
are not donating it to help someone get rich."


reminds me of;

please skroll down a bit to;

some things of interest?

AATB 6th Annual Meeting, March 24-26, 2002 - Slide Presentation

Microbial Contamination and Cross Contamination Concerns During
Processing of Tissue:
an FDA Perspective

Mary Malarkey, Director,


From: TSS (
Subject: re-The Eyes Have It (cjd) and they could be stealing them from
your loved one...
Date: September 17, 2000 at 10:06 am PST

Subject: RE-The Eyes Have It (cjd) and they could be stealing them from
your loved one... "pay back time"
Date: Sat, 16 Sep 2000 10:04:26 -0700
From: "Terry S. Singeltary Sr."
Reply-To: Bovine Spongiform Encephalopathy

Greetings List Members,

I hate to keep kicking a madcow, but this still is very disturbing
to me. Not only for the recipient of the cornea's, but as well, for
the people whom would be operated on, using the same tools that
were used to put those stolen cornea's in the recipient with.
No history of this donor or his family (re-ffi), or anything
would be known, using stolen organs and or tissue's. I just think
this is not only wrong, but very dangerous to a great many other
people, as this is one of the most infectious tissues of TSE's. It seems
that this practice of stealing organ/tissue happens more than we think.
Anyway, the family of the victim which had their cornea's stolen, are
now suing. In the example I used with my Mother, if 3 months before, she
would have been in a catastrophic accident (car wreck, whatever), no
autopsy (for whatever reason), no family (for whatever reason), she lay
in the morgue, and after 4 hours, they come steal the cornea's, lot of
people could have been infected, just because of lack of medical history
of donor/family. It may be hypothetical, but very real. We need to stop
the spread of this disease.

kind regards,
Terry S. Singeltary Sr., Bacliff, Texas USA

Previous story--

Cadaver corneal transplants -- without family permission...


Sept. 15, 2000, 11:39PM


Eye Procedure Raises Cjd Concerns

snip...full text

CJD and intraocular surgery

Ophthalmic surgery and Creutzfeldt-
Jakob disease



Sec. 693.012. Removal of Corneal Tissue Permitted Under Certain


Journal of Virology, February 2005, p. 1888-1897, Vol. 79, No. 3
0022-538X/05/$08.00+0 DOI: 10.1128/JVI.79.3.1888-1897.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.
Neuroinvasion by Scrapie following Inoculation via the Skin Is
Independent of Migratory Langerhans Cells
Joanne Mohan, Moira E. Bruce, and Neil A. Mabbott*

Neuropathogenesis Unit, Institute for Animal Health, Edinburgh,
Scotland, United Kingdom

Received 18 June 2004/ Accepted 7 September 2004

Many natural transmissible spongiform encephalopathy (TSE) infections
are likely to be acquired peripherally, and studies in mice show that
skin scarification is an effective means of scrapie transmission. After
peripheral exposure, TSE agents usually accumulate in lymphoid tissues
before spreading to the brain. The mechanisms of TSE transport to
lymphoid tissues are not known. Langerhans cells (LCs) reside in the
epidermis and migrate to the draining lymph node after encountering
antigen. To investigate the potential role of LCs in scrapie
transportation from the skin, we utilized mouse models in which their
migration was blocked either due to CD40 ligand deficiency (CD40L–/–
mice) or after caspase-1 inhibition. We show that the early accumulation
of scrapie infectivity in the draining lymph node and subsequent
neuroinvasion was not impaired in mice with blocked LC migration. Thus,
LCs are not involved in TSE transport from the skin. After intracerebral
inoculation with scrapie, wild-type mice and CD40L–/– mice develop
clinical disease with similar incubation periods. However, after
inoculation via skin scarification CD40L–/– mice develop disease
significantly earlier than do wild-type mice. The shorter incubation
period in CD40L–/– mice is unexpected and suggests that a
CD40L-dependent mechanism is involved in impeding scrapie pathogenesis.
In vitro studies demonstrated that LCs have the potential to acquire and
degrade protease-resistant prion protein, which is thought to be a
component of the infectious agent. Taken together, these data suggest
that LCs are not involved in scrapie transport to draining lymphoid
tissues but might have the potential to degrade scrapie in the skin.

* Corresponding author. Mailing address: Institute for Animal Health,
Neuropathogenesis Unit, Ogston Bldg., West Mains Rd., Edinburgh EH9 3JF,
United Kingdom. Phone: 44(0)131-667-5204. Fax: 44(0)131-668-3872.

Journal of Virology, February 2005, p. 1888-1897, Vol. 79, No. 3
0022-538X/05/$08.00+0 DOI: 10.1128/JVI.79.3.1888-1897.2005
Copyright © 2005, American Society for Microbiology. All Rights Reserved.

Scrapie transmission following exposure through the skin is
dependent on follicular dendritic cells in lymphoid tissues

Joanne Mohan, Karen L. Brown, Christine F. Farquhar, Moira E. Bruce and
Neil A. MabbottCorresponding Author Contact Information

E-mail The Corresponding Author

Institute for Animal Health, Ogston Building, West Mains Road, Edinburgh

Received 9 March 2004; Revised 22 April 2004; accepted 12 May 2004.
Available online 8 July 2004.


Background: Transmissible spongiform encephalopathies (TSEs) are chronic
infectious neurodegenerative diseases that are characterized by the
accumulation in affected tissues of PrPSc, an abnormal isoform of the
host prion protein (PrPc). Following peripheral exposure, PrPSc usually
accumulates on follicular dendritic cells (FDCS) in lymphoid tissues
before neuroinvasion. Studies in mice have shown that TSE exposure
through scarified skin is an effective means of transmission. Following
inoculation via the skin, a functional immune system is critical for the
transmission of scrapie to the brain as severe combined immunodeficiency
(SCID) mice are refractory to infection. Until now, it was not known
which components of the immune system are required for efficient scrapie
neuroinvasion following skin scarification. Objective: To determine
which cells are critical for the transmission of scrapie to the brain
following inoculation via the skin. Methods: A chimeric mouse model was
used, which had a mismatch in PrPc expression between FDCs and other
bone marrow-derived cells within lymphoid tissues. These chimeric mice
were challenged with scrapie by skin scarification to allow the separate
roles of FDCs and lymphocytes in peripheral scrapie pathogenesis to be
determined. Results: We show that mature FDCs are essential for the
accumulation of scrapie within lymphoid tissues and the subsequent
transmission of infection to the brain following TSE exposure by this
route. Furthermore, we show that the accumulation of PrPSc and
infectivity in the spleen is independent of PrP expression by
lymphocytes or other bone marrow-derived cells. Conclusion: Following
inoculation with scrapie by skin scarification, replication in the
spleen and subsequent neuroinvasion is critically dependent upon mature

Author Keywords: Transmissible spongiform encephalopathy; Scrapie; Skin;
Follicular dendritic cell; Prion protein; Spleen

Corresponding Author Contact Information

author. Tel.: +44 131 667 5204; fax: +44 131 668 3872.

Extraneural Pathologic Prion Protein in Sporadic Creutzfeldt-Jakob Disease


Conclusions Using sensitive techniques, we identified extraneural
deposition of PrPSc in
spleen and muscle samples from approximately one third of patients who
died with
sporadic Creutzfeldt-Jakob disease. Extraneural PrPSc appears
to correlate with a long duration of disease.

Prions in skeletal muscle (Prusiner et al)

> The Belgian cow's results were:
> SAF -
> HP -
> IHC -
> WB +

NOTHING, this is part of june 2004 usda enhanced bse/tse cover-up.
part of the program was to start NOT confirming with WB,
due to the first confirmed finding. same with the other mad cows
in TEXAS i.e. the stumbling and staggering one they refused
to test and rendered;

IF we look at this Belgium atypical cow (and try to forget about those
damn imported belgium sheep that were never confirmed with mouse
bio assay, as we were told they would be, which could very well have
been BSE, i mean there was a declaration of emergency declared for
an ATYPICAL TSE in them), and then look at the #8 and #9 cow of Japan;

8. 6/10/2003 Holstein Steer 13/10/2001 23 mths
No clinical signs WB+, IHC-, HP-

9. 4/11/2003 Holstein Steer 13/1/2002
21 mths No clinical signs WB+, IHC-, HP-

THIS explains very well why the USDA decided to NOT use WB anymore.
damn thing finds things when people don't want it found. simple as that.;f=12;t=000385

Last modified, 11/09/2004 13:42:49

BSE death cow's anomalous prion detected from peripheral nerve tissue,
suprarenal gland

First time from non-Specified Risk Material, or SRM


National Institute of Animal Health Animal announced on November 1 that
it had detected the anomalous prion protein that was the etiologic agent
of the mad cow disease, or BSE, or bovine spongiform encephaalopathy,
from the peripheral nerve tissue and the suprarenal gland of the cow of
the age in the mad cow disease for the dying infection 94 months on
March 9 this year.
Japan is obligating the removal of the Specified Risk Material, or SRM
such as the head, the spinal cord, the vertebral columns, and the small
intestines that accumulate the anomalous prion protein easily as a BSE
(bovine spongiform encephaalopathy) measures.
Because the mad cow disease etiologic agent was detected from a tissue
different from the Specified Risk Material, or SRM, the review of the
Specified Risk Material, or SRM might be urged on the Japanese Government.
International Symposium of PRION DISEASES for food and drug safety
national institute of animal health(only in Japanese)
The statement of the Ministry of Health, Labour and Welfare
(only in Japanese)
Yomiuri on line (only in Japanese)
Asahi on line(only in Japanese)
Mainichi on line(only in Japanese)


Bovine spongiform encephalopathy (BSE) in Japan

Takashi Yokoyama, Kumiko M. Kimura, Morikazu Shinagawa
Prion Disease Research Center, National Institute of Animal Health, Japan

Bovine spongiform encephalopathy (BSE) has become an important problem
not only for animal industry, but
also for public health. In Japan, BSE was first recognized in September
2001 by fallen stock surveillance.
Since October 2001, BSE examination for all cattle slaughtered at
abattoirs has started. In April 2004, all dead
cattle examination (over 24 months) has been conducted at livestock
hygiene service center. Samples positive
in enzyme linked immunosorbent assay (ELISA) are further subjected to
western blot (WB) and
immunohistochemistry (IHC). Thirteen BSE cases have been reported by
September 2004. Twelve cases
were classified as typical BSE, and the remained one was an atypical
BSE. Variant forms of BSE with atypical
histopathological and/or biochemical phenotype were reported in Italy
and France. Further study is required
for BSE prion characteristics.
To characterize BSE prion properties, brain homogenates of Japanese BSE
cases were intracerebrally
inoculated into wild-type mice. The first case (BSE/Chiba) was
successfully transmitted to rodents. The mean
incubation periods (409.0 days) in this experiment was preferably longer
than that of previously reported.
PrPSc distribution, prion titer, mice susceptibility and/or storage
condition of sample might be influenced the
result. Recently, we introduced transgenic mice that overexpress a
bovine PrP gene to overcome the species
barrier problem. These mice are expected to accelerate the transmission
experiment of BSE prion.
Transmission of atypical BSE case is undergoing by using these
transgenic mice.

Research Foundation

Tissue distribution of protease resistant prion protein in variant
Creutzfeldt-Jakob disease using a highly sensitive immunoblotting assay.

Wadsworth JD, Joiner S, Hill AF, Campbell TA, Desbruslais M, Luthert
PJ, Collinge J.

MRC Prion Unit and Department of Neurogenetics, Imperial College
School of Medicine at St Mary's, Norfolk Place, W2 1PG, London, UK.

BACKGROUND: Variant Creutzfeldt-Jakob disease (vCJD) has a
pathogenesis distinct from other forms of human prion disease:
disease-related prion protein (PrP(Sc)) is readily detectable in
lymphoreticular tissues. Quantitation of risk of secondary
transmission, and targeting of risk reduction strategies, is limited
by lack of knowledge about relative prion titres in these and other
peripheral tissues, the unknown prevalence of preclinical vCJD, and
a transmission barrier which limits the sensitivity of bioassay. We
aimed to improve immunoblotting methods for high sensitivity
detection of PrP(Sc) to investigate the distribution of PrP(Sc) in a
range of vCJD tissues. METHODS: We obtained tissues at necropsy from
four patients with neuropathologically confirmed vCJD and from
individuals without neurological disease. Tissues were analysed by
sodium phosphotungstic acid precipitation of PrP(Sc) and western
blotting using high sensitivity enhanced chemiluminescence.
FINDINGS: We could reliably detect PrP(Sc) in the equivalent of 50
nL 10% vCJD brain homogenate, with a maximum limit of detection
equivalent to 5 nl. PrP(Sc) could be detected in tissue homogenates
when present at concentrations 10(4)-10(5) fold lower than those
reported in brain. Tonsil, spleen, and lymph node were uniformly
positive for PrP(Sc) at concentrations in the range of 0.1-15% of
those found in brain: the highest concentrations were consistently
seen in tonsil. PrP(Sc) was readily detected in the retina and
proximal optic nerve of vCJD eye at levels of 2.5 and 25%,
respectively of those found in brain. Other peripheral tissues
studied were negative for PrP(Sc) with the exception of low
concentrations in rectum, adrenal gland, and thymus from a single
patient with vCJD. vCJD appendix and blood (Buffy coat fraction)
were negative for PrP(Sc) at this level of assay sensitivity.
INTERPRETATION: We have developed a highly sensitive immunoblot
method for detection of PrP(Sc) in vCJD tissues that can be used to
provide an upper limit on PrP(Sc) concentrations in peripheral
tissues, including blood, to inform risk assessment models. Rectal
and other gastrointestinal tissues should be further investigated to
assess risk of iatrogenic transmission via biopsy instruments.
Ophthalmic surgical instruments used in procedures involving optic
nerve and the posterior segment of the eye, in particular the
retina, might represent a potential risk for iatrogenic transmission
of vCJD. Tonsil is the tissue of choice for diagnostic biopsy and
for population screening of surgical tissues to assess prevalence of
preclinical vCJD infection within the UK and other populations.

PMID: 11476832 [PubMed - indexed for MEDLINE]

Creutzfeldt-Jakob disease and inclusion body myositis: Abundant
disease-associated prion protein in muscle
Gabor G. Kovacs, MD PhD 1 2, Elisabeth Lindeck-Pozza, MD 1, Leila
Chimelli, MD, PhD 3, Abelardo Q. C. Araújo, MD, PhD 4, Alberto A.
Gabbai, MD, PhD 5, Thomas Ströbel, PhD 1, Markus Glatzel, MD 6, Adriano
Aguzzi, MD, PhD 6, Herbert Budka, MD 1 *
1Institute of Neurology, University of Vienna, and Austrian Reference
Centre for Human Prion Diseases, Vienna, Austria
2National Institute of Psychiatry and Neurology, Budapest, Hungary
3Department of Pathology, School of Medicine, Federal University of Rio
de Janeiro
4Department of Neurology, School of Medicine, Federal University of Rio
de Janeiro
5Department of Neurology, School of Medicine, Federal University of Sao
Paulo, Brazil
6Institute of Neuropathology, University Hospital of Zürich, Zürich,
email: Herbert Budka (

*Correspondence to Herbert Budka, Institute of Neurology, AKH 4J,
Wühringer Gürtel 18-20, POB 48, A-1097 Vienna, Austria

Funded by:
European Union (EU) Project; Grant Number: TSELAB QLK2-CT-2002-81523
EU Concerted Action PRIONET; Grant Number: QLK2-2000-CT-00837


Pathologicalprion protein (PrPSc) is the hallmark of prion diseases
affecting primarily the central nervous system. Using
immunohistochemistry, paraffin-embedded tissue blot, and Western blot,
we demonstrated abundant PrPSc in the muscle of a patient with sporadic
Creutzfeldt-Jakob disease and inclusion body myositis. Extraneural
PrPC-PrPSc conversion in Creutzfeldt-Jakob disease appears to become
prominent when PrPC is abundantly available as substrate, as in
inclusion body myositis muscle.

Received: 16 June 2003; Revised: 11 September 2003; Accepted: 11
September 2003
Digital Object Identifier (DOI)

10.1002/ana.10813 About DOI

NINDS Inclusion Body Myositis Information Page

AS Professor Aguzzi kindly put it most recently ;

Vet Pathol 42:107 108 (2005)
Letters to the Editor
Absence of evidence is not always evidence of absence.
In the article Failure to detect prion protein (PrPres) by
immunohistochemistry in striated muscle tissues of animals
experimentally inoculated with agents of transmissible spongiform
encephalopathy, recently published in Veterinary
Pathology (41:78 81, 2004), PrPres was not detected in striated
muscle of experimentally infected elk, cattle, sheep, and
raccoons by immunohistochemistry (IHC). Negative IHC,
however, does not exclude the presence of PrPSc. For example,
PrPres was detected in skeletal muscle in 8 of 32
humans with the prion disease, sporadic Creutzfeldt-Jakob
disease (CJD), using sodium phosphotungstic acid (NaPTA)
precipitation and western blot.1 The NaPTA precipitation,
described by Wadsworth et al.,3 concentrates the abnormal
isoform of the prion, PrPres, from a large tissue homogenate
volume before western blotting. This technique has increased
the sensitivity of the western blot up to three orders
of magnitude and could be included in assays to detect
PrPres. Extremely conspicuous deposits of PrPres in muscle
were detected by IHC in a recent case report of an individual
with inclusion body myositis and CJD.2 Here, PrPres was
detected in the muscle by immunoblotting, IHC, and paraf-
fin-embedded tissue blot. We would therefore caution that,
in addition to IHC, highly sensitive biochemical assays and
bioassays of muscle are needed to assess the presence or
absence of prions from muscle in experimental and natural
TSE cases.
Christina Sigurdson, Markus Glatzel, and Adriano Aguzzi
Institute of Neuropathology
University Hospital of Zurich
Zurich, Switzerland
1 Glatzel M, Abela E, et al: Extraneural pathologic prion
protein in sporadic Creutzfeldt-Jakob disease. N Engl J
Med 349(19):1812 1820, 2003
2 Kovacs GG, Lindeck-Pozza E, et al: Creutzfeldt-Jakob
disease and inclusion body myositis: abundant diseaseassociated
prion protein in muscle. Ann Neurol 55(1):
121 125, 2004
3 Wadsworth JDF, Joiner S, et al: Tissue distribution of protease
resistant prion protein in variant CJD using a highly
sensitive immuno-blotting assay. Lancet 358:171 180,

EMBO reports AOP Published online: 11 April 2003 Widespread PrPSc
accumulation in muscles of hamsters orally infected with scrapie

2004N-0257: Recordkeeping Requirements for Human Food and Cosmetics
Manufactured from Processed with, or Otherwise Containing Material from

Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA


CJD Watch message board

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