Donated Human Tissues
May Contain
CJD/Mad Cow Prions
Families Of Donors Rarely Get Full Story
Collected Tissue May Be Used In Cosmetic Surgery

By Anne Belli
c. 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 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 donate.
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 fees.
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 reports.
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 processing."
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 corneas, 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. E-mail:
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 FDCs.
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:
HP -
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.
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, Switzerland
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 Cattle
Terry S. Singeltary Sr.
P.O. Box 42
Bacliff, Texas USA



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