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GROWTH-RELATED TRANSGENES Early transgenic farm animal research was inspired by the dramatic growth of transgenic mice that expressed a growth hormone GH transgene.[1] A number of trans-

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Transgenic Animals: Improved Performance

Vernon G Pursel

United States Department of Agriculture, Agricultural Research Service, Beltsville, Maryland, U.S.A

INTRODUCTION

With the world’s population increasing by more than 70

million people each year, modern agricultural methods

that include animal biotechnology will need to be adopted

if this ever-increasing population is going to avoid

massive conflict over agricultural resources The ability

to isolate, clone, and transfer individual genes into farm

animals provides the opportunity for scientists to produce

transgenic animals with modified traits that are

unattain-able through genetic selection

This article reviews progress on transfer of genes for

productivity traits into farm animals, and some areas that

offer promise for the future

GROWTH-RELATED TRANSGENES

Early transgenic farm animal research was inspired by the

dramatic growth of transgenic mice that expressed a

growth hormone (GH) transgene.[1] A number of

trans-genic pigs and sheep were subsequently produced with

human, bovine, rat, porcine, or ovine GH under the

con-trol of several gene promoters.[2]Although pigs

express-ing GH transgenes grew faster, utilized feed more

efficiently, and were much leaner than their

nontrans-genic siblings, they were not larger and exhibited several

notable health problems, which included lameness,

sus-ceptibility to stress, gastric ulcers, and reproductive

prob-lems.[2]The GH transgenic lambs did not grow faster or

utilize feed more efficiently than control lambs, but they

were much leaner and had serious health problems.[2]

More recently, an insulin-like growth factor-I (IGF-I)

transgene has been used to produce transgenic pigs with

enhanced muscle development and reduced fat in the

carcass, but the transgene did not improve growth rate or

feed efficiency In contrast to the GH transgenic pigs,

definitive phenotypes for the IGF-I transgenic pigs were

not detected, and no gross abnormalities, pathologies, or

health-related problems were encountered.[3]

MODIFICATION OF MILK COMPOSITION

Transfer of genes to alter milk composition has thus far

received little research emphasis, but offers the dairy

industry considerable potential for the future A list of potential changes in milk components worthy of consid-eration is shown in Table 1

About 80% of milk protein from cows is composed of caseins (S1, S2, and k), and whey proteins (b

 -lactoglob-ulin, a

-lactalbumin, serum albumin, and g-globulin) make

up the remaining 20%

The caseins form the curds in cheese, whereas the whey proteins represent a less valuable by-product Elimination of b

-lactoglobulin from milk would benefit cheese production because it inhibits rennin’s action on k-casein,[4]and would benefit certain fluid milk consumers because b

-lactoglobulin is responsible for some milk allergies Removal of b

-lactoglobulin from cattle is now technically feasible during transfection of fetal fibroblasts that are then used for nuclear transfer.[7]

While removal of a-lactalbumin (a-lac) from cows’ milk may be beneficial for some consumers, researchers at the University of Illinois have shown that increased concentrations of lactose, which result from a

-lac expression, may be beneficial for piglet growth.[8] They produced transgenic pigs that express bovine a

 -lactalbu-min in their milk, which results in a higher milk lactose content in early lactation and a 20 to 50% greater milk yield on days 3 9 of lactation, compared to that of control sows Weight gain of piglets suckling a-lac sows was greater at days 7 and 21 after parturition than that of control piglets Thus, overexpression of a-lac milk protein provides a means for improving growth performance of piglets through enhanced lactation of sows

WOOL PRODUCTION

Three transgenic approaches have been investigated for enhancing wool production or improving wool quality The first involved transfer of bacterial genes that had the capacity to synthesize cysteine from hydrogen sulfide and serine, both of which are available in the rumen Cysteine

is the rate-limiting amino acid for wool production, so an endogenous source of this amino acid has the potential to stimulate wool growth The second approach to improve wool production was to stimulate fiber growth by expression of an IGF-I transgene specifically in wool follicles The third approach was to improve wool fiber quality by altering expression of wool fiber keratin and

DOI: 10.1081/E EAS 120019824

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keratin-associated protein genes in the wool follicle

cortex.[9] Research on the latter approach is still

under-way in South Australia

ENHANCED ANIMAL HEALTH

Economic losses from diseases of farm animals have been

estimated to amount to 10 to 20% of the total production

costs Use of transgenesis in farm animals holds great

promise for augmenting conventional breeding techniques

to confer animals with improved resistance to these diseases and thereby reduce these losses and enhance animal welfare Unfortunately, most of the genes involved

in disease resistance or susceptibility to disease are still largely unknown In addition to naturally occurring resistance genes, transgenes could be composed of genes that enhance immune response or in vitro-designed gene products (Table 2) Several approaches that have been investigated include transfer of genes for providing

Table 2 Naturally occurring disease resistance/susceptibility genes and in vitro designed genes conferring resistance

Encoding receptors for pathogens Antimicrobial peptides

Nonspecific immunity Enhancing the level and type of the immune response

(e.g., chemokines and cytokines) Encoding complement proteins Regulating phagocyte uptake and killing (e.g., NOS, Nramp) Specific (acquired) immunity Encoding receptors binding directly or indirectly to antigens

(T cell receptors, immunoglobulins, major histocompatibility complex, etc.) Mechanism Immunization (i.e., antibody production) DNA vaccines, immunoglobulin cDNAs

Interference with pathogen entry Recombinant pathogen receptors, coreceptors, etc

Interference with pathogen replication Antisense RNA, ribozymes, intrabodies (Source: Ref 10.)

Table 1 Some proposed modifications of milk constituents

Increase a and b

thermal stability, and increased calcium content Increase phosphorylation sites in caseins Increased calcium content,

improved emulsification Introduce proteolytic sites in caseins Increased rate of textural development to

improve cheese ripening

decreased micelle size, decreased gelation and coagulation Eliminate b

improved digestibility, decreased allergenic response, decreased primary source of cysteine in milk

decreased ice crystal formation, compromised osmotic regulation of mammary gland

and increased cheese yield

Decrease expression of acetyl CoA carboxylase Decreased fat content, improved nutritional quality,

reduced milk production costs Express immunoglobulin genes Protection against pathogens such as salmonella and listeria Replace bovine milk protein genes with human equivalents Mimic human breast milk

(Source: Refs 4 6.)

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resistance to influenza in pigs, preformed antibodies in

pigs, viral envelope proteins in chickens and pigs, and

antimicrobial peptides.[10]

As a first step toward enhancing mastitis resistance of

dairy animals, researchers generated transgenic mice that

secrete a potent antistaphylococcal protein, lysostaphin,

into milk.[11] Lysostaphin is a peptidoglycan hydrolase

normally produced by Staphylococcus simulans that is

active against Staphylococcus aureus bacteria S aureus

is the major contagious mastitis pathogen, accounting for

more than 15% of mastitis infections, and has proved

difficult to control using standard management practices

Three lines of transgenic mice were produced with an

ovine b

-lactoglobulin gene directing the secretion of

lysostaphin into milk Progeny of these mice exhibited

substantial resistance to an intramammary challenge of

S aureus, with the highest expressing line being

com-pletely resistant to infection These results clearly

demon-strated the potential of a transgene to combat one of the

most prevalent diseases of dairy cattle The same

lyso-staphin transgene has now been used to produce

trans-genic dairy cattle that are currently being evaluated

REDUCED ENVIRONMENTAL POLLUTION

In an effort to reduce phosphorus excretion in swine

manure, researchers at the University of Guelph[12]

constructed a transgene to provide expression of phytase

in salivary glands of pigs The saliva of these pigs contains

the phytase enzyme that allows the pigs to digest the

phosphorus in phytate, which is the most abundant source

of phosphorus in the pig diet Without this enzyme,

phosphorus in phytate passes undigested into feces to

become the single most important pollutant of swine

manure Their research showed that salivary phytase

essentially provides complete digestion of dietary phytate

phosphorus, relieves the requirement for inorganic

phosphate supplements, and reduces fecal phosphorus

output by up to 75% These pigs offer a unique biological

approach to the management of phosphorus nutrition and

reduce one of the major environmental pollutants

generated on swine farms

CONCLUSION

In the past few years, transgenic research to alter carcass

composition, increase milk production in sows, enhance

disease resistance, and reduce excretion of phosphate in

pigs has shown substantial progress Modification of milk

composition traits in dairy cattle offers considerable

potential, but much of this research is dependent upon

improving the efficiency of nuclear transfer, which will distinctly reduce the cost of producing transgenic cattle

REFERENCES

1 Palmiter, R.D.; Brinster, R.L.; Hammer, R.E.; Trumbauer, M.E.; Rosenfeld, M.G.; Birnberg, N.C.; Evans, R.M Dramatic growth of mice that develop from eggs micro injected with metallothionein growth hormone fusion genes Nature 1982, 300, 611 615

2 Pursel, V.G.; Rexroad, C.E., Jr Status of research with transgenic farm animals J Anim Sci 1993, 71 (Suppl 3),

10 19

3 Pursel, V.G.; Mitchell, A.D.; Wall, R.J.; Solomon, M.B.; Coleman, M.E.; Schwartz, R.J Transgenic Research to Enhance Growth and Lean Carcass Composition in Swine

In Molecular Farming; Toutant, J.P., Balazs, E., Eds.; INRA: Paris, 2001; 77 86

4 Jimenez Flores, R.; Richardson, T Genetic engineering of the caseins to modify the behavior of milk during processing: A review J Dairy Sci 1985, 71, 2640 2654

5 Yom, H C.; Bremel, R.D Genetic engineering of milk composition: Modification of milk components in lactating transgenic animals Am J Clin Nutr 1993, 58 (Suppl),

299 306

6 Maga, E.A.; Murray, J.D Mammary gland expression of transgenes and the potential for altering the properties of milk Bio/Technology 1995, 13, 1452 1457

7 Denning, C.; Burl, S.; Ainslie, A.; Bracken, J.; Dinnyes, A.; Fletcher, J.; King, T.; Ritchie, M.; Ritchie, W.A.; Rollo, M.; de Sousa, P.; Travers, A.; Wilmut, I.; Clark, A.J Deletion of the alpha (1,3) galactosyl transferase (GGTA1) gene and the prion protein (PrP) gene in sheep Nat Biotechnol 2001, 19, 559 562

8 Noble, M.S.; Rodriguez Zas, S.; Cook, J.B.; Bleck, G.T.; Hurley, W.L.; Wheeler, M.B Lactational performance of first parity transgenic gilts expressing bovine alpha lactalbumin in their milk J Anim Sci 2002, 80, 1090 1096

9 Bawden, C.S.; McLaughlan, C.J.; Walker, S.K.; Speck, P.A.; Powell, B.C.; Huson, M.J.; Jones, L.N.; Rogers, G.E Improvement of Wool Quality by Transgenesis In Molecular Farming; Toutant, J.P., Balazs, E., Eds.; INRA: Paris, 2001; 67 76

10 Mu¨ller, M Increasing Disease Resistance in Transgenic Domestic Animals In Molecular Farming; Toutant, J.P., Balazs, E., Eds.; INRA: Paris, 2001; 87 97

11 Kerr, D.E.; Plaut, K.; Bramley, A.J.; Williamson, C.M.; Lax, A.J.; Moore, K.; Wells, K.D.; Wall, R.J Lysostaphin expression in mammary glands confers protection against staphylococcal infection in transgenic mice Nat Biotech nol 2001, 19, 66 70

12 Golovan, S.P.; Meidinger, R.G.; Ajakaiye, A.; Cottrill, M.; Wiederkehr, M.Z.; Barney, D.J.; Plante, C.; Pollard, J.W.; Fan, M.Z.; Hayes, M.A.; Laursen, J.; Hjorth, J.P.; Hacker, R.R.; Phillips, J.P.; Forsberg, C.W Pigs expressing salivary phytase produce low phosphorus manure Nat Biotechnol 2001, 19, 741 745

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Transgenic Animals: Modifying the

Mitochondrial Genome

Carl A Pinkert

University of Rochester Medical Center, Rochester, New York, U.S.A

Lawrence C Smith

Universite´ de Montre´al, Quebec, Canada

Ian A Trounce

University of Melbourne, Victoria, Australia

INTRODUCTION

In comparison to the techniques successfully employed

for nuclear gene transgenesis in livestock over the past 20

years, the lack of comparable recombination in

mitochon-drial DNA (mtDNA) has, until recently, prevented its

direct in vivo manipulation The coordinated expression

of single-copy nuclear gene products, together with the

polyploid mtDNA gene products, is required for normal

mitochondrial biogenesis and respiratory chain function

It is of great current interest to seek improved

technol-ogies for manipulating the mitochondrial genome, so that

interactions of nuclear and mtDNA genotypes can be

studied in experimental systems

MITOCHONDRIAL GENETICS

AND ANIMAL MODELING

Mammalian mitochondria contain between one and

approximately ten copies of a closed, circular,

super-coiled, double-stranded DNA that is bound to the inner

mitochondrial membrane and is not associated with

histones or a scaffolding protein matrix The mtDNAs of

all vertebrates are highly conserved and quite small

( 16.5 kb in length) in comparison to the nuclear

genome Mammalian mitochondria have their own genetic

systems, replete with a unique genetic code, genome

structure, transcriptional and translational apparatus, and

tRNAs Perhaps, because of a postulated less-extensive

mitochondrial DNA (mtDNA) repair system and because

of the absence of protective histones, the mitochondrial

genome is subject to an increased sensitivity to mutations

due to metabolic (e.g., oxidative stress) and environmental

(e.g., toxins, mutagens, and UV light) sources

Mitochon-drial genes encode for 13 of the protein subunits that

function in the mitochondrial oxidative phosphorylation

system, along with two ribosomal RNAs (rRNAs) and 22 transfer RNAs (tRNAs) Accordingly, directed modifica-tion of mitochondrial genes and/or their funcmodifica-tion would provide a powerful tool in production agriculture.[1] Cytoplasmic-based traits in domestic animals have included growth, reproduction, and lactation In addition, mitochondrial restriction fragment-length polymorphisms (RFLPs) were identified and associated with specific lactational characteristics in a number of dairy cattle lineages The matrilineal inheritance of mammalian mtDNA has also been used to advantage in studies exploring the timing and geography of domestication events, as recently demonstrated for horses, where multiple domestication events appear to have occurred in the Eurasian steppe.[2] In addition, metabolic and cellular abnormalities in humans were correlated to mutations arising exclusively within the mitochondrial genome Indeed, various diseases have been associated with mtDNA point mutations, deletions, and duplications (e.g., diabetes mellitus, myocardiopathy, and retinitis pigmentosa) as well

as age-associated changes in the functional integrity of mitochondria (as seen in Parkinson’s, Alzheimer’s, and Huntington’s diseases) As such, for both agricultural and biomedical research efforts, the ability to manipulate the mitochondrial genome and to regulate the expression of mitochondrial genes would provide one possible mode of genetic manipulation and therapy

The creation of heteroplasmic transmitochondrial animals has developed along three lines: 1) direct mito-chondrial injection into oocytes or embryos; 2) embryonic stem (ES) cell-based technologies; and 3) in relation to karyoplast or cytoplast transfer (including consequences associated with nuclear transfer or cloning experimenta-tion; Table 1) These techniques have illustrated model systems that will provide a greater understanding of mito-chondrial dynamics, leading to the development of ge-netically engineered production animals, and therapeutic

DOI: 10.1081/E EAS 120024365

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strategies for human metabolic diseases affected by

aberrations in mitochondrial function

As described in a number of recent reports,[3,4]

nuclear-encoded genes and knock-out modeling have been

informative in identifying novel models in mitochondrial

disease pathogenesis as well as critical pathways

associ-ated with mitochondrial function With initial

character-ization of these nuclear gene-encoded models, our search

for a greater understanding of mitochondrial interactions

and function would eventually lead us to a desire to

develop methodology for mitochondria and mitochondrial

gene transfer As a first step, efficient methods to

introduce foreign or altered mtDNA or genomes into

somatic or germ cells would be needed

TRANSMITOCHONDRIAL ANIMALS

To make a transmitochondrial animal, the ability to

manipulate normal and mutant mitochondria in vivo has

been a critical and difficult first step In vivo

mitochon-drial gene transfer remains a technological hurdle in the

development of mitochondria-based genetic therapies and

in the generation of experimental animal models for the

study of mitochondrial dynamics and mitochondria-based

traits While gene transfer has been performed in a host of

cell types and organisms, transfer of nuclear DNA has

been the only demonstrable form of mammalian gene

transfer, short of cell fusions, to date

Rapid segregation of mtDNA genotypes could occur in

mammals and was first demonstrated in Holstein cattle

where pedigree records in the industry allowed detailed

analysis of maternally related individual genotypes.[5]

Segregation of mtDNA was investigated in maternal

lineages of heteroplasmic mice created by cytoplast

fusion[6,7]and by embryonic karyoplast transplantation.[8]

Although mitochondrial segregation in somatic tissues is

effective in some tissues and with increasing age, the preceding studies have shown that mtDNA heteroplasmy

is maintained at stable levels throughout several gen-erations This would suggest that the mouse germline

is not very effective in segregating mtDNA haplotypes

In cattle, however, highly heteroplasmic females will produce homoplasmic oocytes, whereas heteroplasmic bulls produce mostly heteroplasmic sperm, indicating that mtDNA segregation is very stringent in the female and practically absent in the male germline.[9]Together, these results suggest that mammalian species show variable patterns of mtDNA segregation

In contrast to these techniques, our efforts to devise a direct mitochondria transfer technique offered certain advantages Principally, the ability to use isolated mito-chondria for the production of heteroplasmic mice would allow for investigations into the feasibility of genetic manipulation of mtDNA in vitro prior to mitochondria microinjection into zygotes

CONCLUSIONS

Through the early 1990s, various early attempts to create transmitochondrial strains of mammalian species by introduction of foreign mitochondria into germ cells were largely unsuccessful A number of constraints have been identified or postulated, from perturbations of biological pathways to mechanistic aspects of the specific protocols used Since 1997, a number of laboratories have reported

on methodologies used to create transmitochondrial animals To date, methods for mitochondria isolation and interspecific transfer of mitochondria have been reported both in laboratory and domestic animal models.[3,10,11] Interestingly, early reports on development of cloned animals by nuclear transfer resulted in conflicting con-sequences when retrospective studies on mitochondrial

Table 1 Methods for creating mitochondrial modifications in animals

Method

Heteroplasmy/

homoplasmy detected

Germline

Karyoplast fusion

(nuclear transfer)

PEG or electrofusion Karyoplast or cytoplast

transfer into ES cells and transfer

competent/efficient cell lines

low level heteroplasmy

or programmed destruction postfertilization

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transmission were reported.[12–16]Indeed, dependent upon

the specific methodology employed for nuclear transfer

and cytoplasm/ooplasm transfer to rescue low-quality

embryos, additional models of heteroplasmy may or may

not have been characterized as a consequence of

mitochondrial dysfunction As such, research independent

of targeted mitochondrial genomic modifications may also

help unlock mechanisms underlying the dynamics related

to persistence of foreign mitochondria and maintenance of

heteroplasmy in various cloning protocols

ARTICLES OF FURTHER INTEREST

Biotechnology: Stem Cell and Germ Cell Technology,

p 146

Biotechnology: Transgenic Animals, p 149

Contributions to Society: Biomedical Research Models,

p 239

Genetics: Molecular, p 466

Molecular Biology: Animal, p 653

Transgenic Animals: Improved Performance, p 837

REFERENCES

1 Pinkert, C.A Genetic Engineering of Animals In Hand

book of Biomedical Technology and Devices; Moore, J.E.,

Jr., Zouridakis, G., Eds.; CRC Press: Boca Raton, 2004;

18 1 18 12

2 Vila, C.; Leonard, J.A.; Gotherstrom, A.; Marklund, S.;

Sandberg, K.; Liden, K.; Wayne, R.K.; Ellegren, H

Widespread origins of domestic horse lineages Science

2001, 291 (5503), 474 477

3 Pinkert, C.A.; Trounce, I.A Production of transmitochon

drial mice Methods 2002, 26 (4), 348 357

4 Wallace, D.C Mouse models for mitochondrial disease

Am J Med Genet 2001, 106 (1), 71 93

5 Olivo, P.D.; Van de Walle, M.J.; Laipis, P.J.; Hauswirth,

W.W Nucleotide sequence evidence for rapid genotypic

shifts in the bovine mitochondrial DNA D loop Nature

1983, 306 (5941), 400 402

6 Jenuth, J.P.; Peterson, A.C.; Fu, K.; Shoubridge, E.A

Random genetic drift in the female germline explains the

rapid segregation of mammalian mitochondrial DNA Nat Genet 1996, 14 (2), 146 151

7 Jenuth, J.P.; Peterson, A.C.; Shoubridge, E.A Tissue specific selection for different mtDNA genotypes in heteroplasmic mice Nat Genet 1997, 16 (1), 93 95

8 Meirelles, F.V.; Smith, L.C Mitochondrial genotype segregation in a mouse heteroplasmic lineage produced

by embryonic karyoplast transplantation Genetics 1997,

145(2), 445 451

9 Smith, L.C.; Bordignon, V.; Garcia, J.M.; Meirelles, F.V Mitochondrial genotype segregation and effects during mammalian development: Applications to biotechnology Theriogenology 2000, 53 (1), 35 46

10 Meirelles, F.V.; Bordignon, V.; Watanabe, Y.; Watanabe, M.; Dayan, A.; Lobo, R.B.; Garcia, J.M.; Smith, L.C Compete replacement of the mitochondrial genotype in a Bos indicus calf reconstructed by nuclear transfer to a Bos taurus oocyte Genetics 2001, 158 (1), 351 356

11 McKenzie, M.; Trounce, I.A.; Cassar, C.A.; Pinkert, C.A Production of homoplasmic xenomitochondrial mice Proc Natl Acad Sci USA 2004, 101 (6), 1685 1690

12 Hiendleder, S.; Zakhartchenko, V.; Wenigerkind, H.; Reichenbach, H.D.; Bruggerhoff, K.; Prelle, K.; Brem, G.; Stojkovic, M.; Wolf, E Heteroplasmy in bovine fetuses produced by intra and inter subspecific somatic cell nuclear transfer: Neutral segregation of nuclear donor mitochondrial DNA in various tissues and evidence for recipient cow mitochondria in fetal blood Biol Reprod

2003, 68 (1), 159 166

13 Evans, M.J.; Gurer, C.; Loike, J.D.; Wilmut, I.; Schnieke, A.E.; Schon, E.A Mitochondrial DNA genotypes in nuclear transfer derived cloned sheep Nat Genet 1999,

23(1), 90 93

14 Hiendleder, S.; Schmutz, S.M.; Erhardt, G.; Green, R.D.; Plante, Y Transmitochondrial differences and varying levels of heteroplasmy in nuclear transfer cloned cattle Mol Reprod Dev 1999, 54 (1), 24 31

15 Steinborn, R.; Schinogl, P.; Zakhartchenko, V.; Achmann, R.; Schernthaner, W.; Stojkovic, M.; Wolf, E.; Muller, M.; Brem, G Mitochondrial DNA heteroplasmy in cloned cattle produced by fetal and adult cell cloning Nat Genet

2000, 25 (3), 255 257

16 Takeda, K.; Takahashi, S.; Onishi, A.; Goto, Y.; Miya zawa, A.; Imai, H Dominant distribution of mitochondrial DNA from recipient oocytes in bovine embryos and offspring after nuclear transfer J Reprod Fertil 1999,

116(2), 253 259

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Transgenic Animals: Secreted Products

Michael J Martin

David A Dunn

Carl A Pinkert

University of Rochester Medical Center, Rochester, New York, U.S.A

INTRODUCTION

Interest in modifying traits that determine productivity of

domestic animals was greatly stimulated by early

experi-ments in which body size and growth rates were

dramatically affected in transgenic mice expressing

growth hormone transgenes driven by a metallothionein

(MT) enhancer/promoter From that starting point, similar

attempts followed to enhance growth in farm animals by

introduction of various growth factors, modulators, and

their receptors It soon became apparent that transgene

regulation was an exquisite balancing act, where precise

regulation of transgenes was crucial to normal

devel-opment Yet, the overexpression of various transgene

products illustrated that such animals could produce

biologically important molecules as efficient mammalian

bioreactors, with efficiencies far greater than conventional

bacterial or cell culture systems From early studies in the

mid-1980s through the 1990s, one of the main targets of

genetic engineering or gene pharming efforts has involved

attempts to direct expression of transgenes encoding

biologically active human proteins in farm animals To

date, expression of foreign genes encoding various protein

products was successfully targeted to the mammary

glands of goats, sheep, cattle, and swine, yet the jump

from model to achieving regulatory approval has proven

most challenging

ADVANTAGES OF RECOMBINANT PROTEIN

SYNTHESIS IN TRANSGENIC ANIMALS

Several different organisms have been harnessed to

produce recombinant proteins Bacteria, yeast, fungi,

plants, and cultured mammalian cells can all be

reprogrammed and, if properly managed, yield relatively

large amounts of recombinant proteins Problems begin to

arise, however, when one examines the ability of these

organisms to posttranslationally modify and even release

recombinant proteins Bacteria, for example, are often

unable to package and secrete recombinant proteins In

these instances, the recombinant protein must be

physi-cally extracted from the bacteria, a process that can be difficult and costly Whereas yeast can secrete recombinant proteins that are glycosylated, the enzymatic pathway(s) that they utilize to accomplish protein glycosylation differs from that employed in higher plants and animals

As a result, many of the recombinant proteins produced

by yeast exhibit inadequate glycosylation Posttrans-lational modification of recombinant proteins produced

in fungi appears to be aberrant in many instances as well Mammalian cell lines, in contrast, typically perform posttranslational modifications of recombinant proteins that are quite similar to those observed in indigenous proteins Primary drawbacks to the synthesis of recom-binant proteins in animal cell lines include cost and the logistical challenge associated with developing and managing cell cultures for large-scale protein production

In contrast, transgenic animals, as Louis-Marie Hou-debine describes,[1] share most of the properties of animal cells in culture, exhibit appropriate posttransla-tional modifications of recombinant proteins, and synthe-size and secrete proteins extremely efficiently Indeed, mammary gland epithelia typically have a cell density that is 100- to 1000-fold greater than that used in mam-malian cell culture bioreactors In one recent example, 35 transgenic goats that produced a human monoclonal antibody at a concentration of 8 g/L in their milk were equivalent to an 8500-liter batch cell culture running

200 days/year with a 1 g/l final production level.[2]Thus, from a production standpoint, the amount of antibody synthesized in 170,000-liter cell culture yield was equiv-alent to that generated in 21,000 liters of milk from transgenic goats Assuming a process yield of 60%, both systems would generate 100 kg of purified monoclonal antibody, yet the transgenic bioreactor was significantly more efficient

Another obvious incentive for the production of biopharmaceuticals in transgenic livestock is their poten-tial economic value (Table 1) The cost of human proteins obtained from donated plasma and used in replacement therapy has ranged from $4/g for serum albumin and

$5000/g for antithrombin III to $150,000/g for human blood clotting factor VIII (FVIII).[4] Although the individual values of these seem dramatic, they pale in

DOI: 10.1081/E EAS 120019825

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comparison to the projected worth of a number of

recombinant structural products Biomedical applications

of BiosteelTM(Nexia Inc.), a recombinant form of dragline

spider silk, produced in the milk of transgenic goats, is

projected to represent $150 to $450 million in annual

earnings (exclusive of military and other

industri-al applications)

EXPRESSION OF RECOMBINANT

PROTEINS IN MILK

Since the introduction of the first exogenous genes into

mice, more than 60 proteins have been produced in milk

of transgenic animals In order to target protein expression

specifically to the mammary gland, a transgene typ-ically consists of the desired protein gene fused to one

of several available mammary-specific regulatory se-quences.[3–7]These sequences have included: ovine BLG; murine, rat, and rabbit whey acidic protein (WAP); bovine

a-s1casein; rat, rabbit, and goatb-casein; and guinea pig, ovine and caprine, and bovine a-lactalbumin While ex-pression of the target protein can be achieved using either a genomic DNA or cDNA coding sequence(s), the former normally yields higher levels of protein expression Therapeutic monoclonal antibodies produced in the mammary gland of a transgenic animal line present a potentially valuable technology Transgenic monoclonal antibodies are produced by cloning genetic sequences for both heavy- and light-chain genes downstream of

Table 1 Molecular pharming projects: Potential biomedical and commercial products from transgenic farm animals

a fetoprotein (rhAFP) Myasthenia gravis, multiple sclerosis,

and rheumatoid arthritis

(Merrimack/GTC)

Phytase (EnviropigTM

Spider silk (Biosteel1

Monoclonal antibodies and immunoglobulin fusion proteins:

AntegrenTM

and rheumatoid arthritis

(Merrimack /GTC)

(Adapted from Ref 3.)

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mammary gland-specific regulatory elements Chimeric

antibodies may also be produced by ligating

antigen-binding region sequences from a (usually murine)

monoclonal antibody to constant region sequences from

a different species and/or isotype The first transgenic

mice harboring immunoglobulin genes were made in the

mid-1980s.[8]Though the majority of effort and funding in

this field is currently focused toward human therapeutics,

veterinary use of monoclonal antibodies also shows

significant promise as a developing application

Whereas several therapeutic monoclonal antibodies

have been approved for use by the U.S Food and Drug

Administration, none as yet has been approved where a

transgenic animal was used as a production vehicle Using

antibody production technologies in transgenic

biore-actor systems, these products target a wide range of

clinical ailments and are mostly in the preclinical stage

of development

EXPRESSION OF RECOMBINANT PROTEINS

IN MEDIA OTHER THAN MILK

Secretion of transgene-encoded proteins in the urine of

transgenic animals was demonstrated using recombinant

genes under the control of kidney-[9] or bladder-[10]

specific regulatory sequences Expression of transgenes in

the kidney or bladder of transgenic animals and

subsequent secretion in the urine may provide some

advantages over the mammary gland as a bioreactor, as

the purification of proteins from urine may be facilitated

by lower lipid and protein levels in comparison to milk

Additionally, such animals can be used for production

of recombinant proteins over the course of their entire

life span

RECOMBINANT PROTEIN PRODUCTION:

HEALTH AND SAFETY ISSUES

In addition to being structurally and functionally

analo-gous to the natural plasma-derived protein, purified

recombinant proteins must be free of pathogenic

organ-isms Viral and bacterial contamination of human

biopharmaceutical products produced in the blood or

milk can be minimized by focusing prevention/eradication

efforts on at least three levels of production: the transgenic

animal donor, the medium in which the recombinant

protein is produced, and the final product.[4]An initial key

to minimizing the risk of contamination is to derive the

transgenic donor animals from a source herd that is free

from as many pathogens as possible Maintenance of these

animals in a closed facility, the implementation of strict

monitoring procedures for various pathogens, and the use

of animal husbandry practices that follow generally accepted practices (GAPs) and standard operating proce-dures should greatly reduce the entry of pathogens Though quite costly, one can develop pathogen-free herds

of transgenic livestock Such a feat was recently achieved

by introducing hysterotomy-derived transgenic piglets into an elaborate SPF barrier facility.[11] Diagnostic testing of this herd over the past 3 years in this facility had revealed the absence of 35 major and minor swine pathogens including PRRS, parvovirus, leptospira, para-influenza, and Streptococcus suis

CONCLUSION

While transgenic animal technology continues to open new and unexplored agricultural frontiers, molecular pharming efforts raise questions concerning regulatory and commercialization issues Although significant advances have been made since the inception of various clinical trials, the resources required to move the projects forward and the attendant financial risks have led a number of companies to curtail product development Various societal issues exist and will continue to influence the development of value-added animal products pro-duced through transgenesis until transgenic products and foodstuffs are proven safe for human use and are accepted by a wide cross section of society

ARTICLES OF FURTHER INTEREST

Biotechnology: Stem Cell and Germ Cell Technology,

p 146 Biotechnology: Transgenic Animals, p 149 Contributions to Society: Biomedical Research Models,

p 239 Genetics: Molecular, p 466 Molecular Biology: Animal, p 653 Overall Contributions of Domestic Animals to Society,

p 696 Phytases, p 704 Proteins, p 757 Transgenic Animals: Improved Performance, p 837

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