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565 A Placebo Controlled Phase IIb Clinical Studyof TissueGene C (TG C) in Patients with Osteoarthritis Molecular Therapy Volume 21, Supplement 1, May 2013 Copyright © The American Society of Gene & C[.]

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Molecular Therapy Volume 21, Supplement 1, May 2013

DNA VECTOROLOGY & GENE TARGETING II

provide a feasible strategy to design and make novel Ad vector for

vaccine development, which will avoid spending too much time and

cost to screen new Ad vectors based on the traditional way

563 Construction of a Novel Bicistronic

Adenoviral Vector

Feilong Jie,1 Shengyao Wang,2 Jie Luo,1 Na Zhang,3 Jingang

Zhang,3 Hongwei Li.1

1 Southern Medical University, Guangzhou, Guangdong,

China; 2 Mulan Pharmaceuticals, Shenzhen, China; 3 Institute of

Transfusion Medicine, The Academy of Military Medical Sciences,

Beijing, China.

Achieving consistent expression of two therapeutic proteins in

the same cells is an important issue for gene therapy applications

Currently, the most commonly employed strategies include the use of

multiple promoters, or internal ribosome entry site (IRES) elements

However, it has shown that the protein encoded downstream of the

IRES in these vectors is not reliably expressed Meanwhile, the

use of multiple promoters may be compromised by interference

between promoters, promoter silencing, and vector rearrangements

or deletions We have constructed a novel adenoviral vector,

pShuttle-CMV-GFP-2A-Cherry, with a shared CMV promoter and a 2A peptide

encoding region from the foot-and-mouth disease virus (FMDV),

which cleaves at its own C-terminus, thereby separating the “fusion

protein” into two independent proteins Importantly, the restriction

enzyme Apa I site at the 5’ end is designed to help replace Cherry

with target proteins, resulting in the C site of cleaved fusion protein

with an excess proline In our study, 293T cells were separately

transfected by the calcium phosphate method with the plasmids

CMV-GFP-2A-Cherry, CMV-GFP, or

pShuttle-CMV-Cherry while 293A cells were separately infected with Ad5-

CMV-GFP-2A-Cherry, Ad5-CMV-GFP or Ad5-CMV-Cherry We

found that GFP or Cherry expression delivered by related plasmids

or packaged viruses were almost at the same level no matter the cell

type It indicates that the novel adenoviral vector can elicit bicistronic

expression simultaneously and equivalently Moreover, the C end of

cleaved fusion protein has only one excess amino acid of proline

These results will greatly benefi t the study on both structure and

function of target protein and gene therapy

564 Ciprofl oxacin Regulates Negatively

Egr-1 Transcriptional Activity in Human Primary

Tenocytes Transduced with Ad-Egr-1/Luc

Francisco Martinez-F,1,2 Araceli Barrera-Lopez,1 Hugo

Sandoval-Zamora,1 Karina Guzman-M,1 Alejandro Jimenez-Orozco,2

Christian Y Lomeli-R,1 David T Curiel,3 Rebecca E

Franco-Bourland.4

1 Molecular Biotherapeutic Program, Skin & Tissue Bank,

National Institute of Rehabilitation Ministry of Health, Mexico

City, DF, Mexico; 2 Dapartment of Pharmacology, School of

Medicine, National Univeristy of Mexico, Mexico City, DF,

Mexico; 3 Department of Radiation Oncology, School of Medicine,

University of Washington., St Louis, MO; 4 Department of

Biochemistry, National Institute of Rehabilitation, Mexico.

Introduction: Tenocytes proliferation rate is an essential factor for

remodeling and maintenance of tendon integrity Negative balance of

the rhythm of cell proliferation is a key factor for infl ammatory process

and tendon rupture Helicases inhibitors such as fl uoroquinolones have

been reported as factor for tendon rupture Hereby, we analyze the

effect of ciprofl oxacin on rate of cell proliferation in human primary

tenocytes and the effect on the transcriptional regulation of egr-1

promoter based on transduction of adenoviral vector Adegr1-Luc

Materials and methods: Cells and adenoviral vectors: No replicative

recombinant adenovirus (AdEgr1-Luc) were packaged at large scale

in HEK-293 cells and purifi ed according to the current protocol based

on cesium chloride gradient protocol for in vivo application Viral Stock was titled by plaque assay and OD Human primary tenocytes (HPT) were obtained based on collagenase digestion protocol and cultured in DMEM/F12 Media supplemented with 10% HI-FBS and antibiotics under standard culture conditions for 1 week and stored for experimental procedure 5x104 Tenocytes were seeded/well After

12 hrs, cells were infected with AdEgr1-Luc at 50 MOI´s during two hrs in serum reduced media After infection, cells were keeped

in 1% of FBS for 24 hours at environment standard conditions for culture and ciprofl oxacin at 5 a 10 μg/ml (15, 30, 60, 90 and 120 seg) Protein extraction was performed at 2, 6 and 12 hrs based

on Cell Glo Lysis Buffer (Promega corp.) and luciferase activity was quantifi ed using a multidetector DTX-880 Results: Human tenocytes transduced with AdEgr1-Luc are positively responsive

to (10%) of Egr-1 promoter (18,233 LC/s) Luminescent activity

in presence of ciprofl oxacin was observed at 2, 6 and 12 hrs (1,490 LC/S; 1,704.66 LC/s and 1,851.33 LC/s, respectively) Conclussion Ciprofl oxacin inhibits transcriptional activity of egr-1 promoter in human primary tenocytes However, this fact is actually studies to determinate the signal transduction of regulation in tenocytes and the effect on cell proliferation rate by Cell proliferation assays Acknowledgments: This research project is granted by the National Council of Science and Technology of México Grant FOSIS/ CONACYT-Salud-2011-1-161624

DNA Vectorology & Gene Targeting II

565 A Placebo Controlled Phase IIb Clinical Studyof TissueGene-C (TG-C) in Patients with Osteoarthritis

Jung Jong Cho,1 Tae Won Kim,1 Yeo Myeong Park,1 Eu Gene Jeong,1 Moon Jong Noh,1 Kwan Hee Lee,1 Bum Sup Lee.1

1 Kolon Life Science, Inc., Gwacheon-Si, Gyeonggi-Do, Korea.

The objective of this study was to determine both safety and effi cacy of TG-C in patients with knee osteoarthritis (OA) TG-C is

a cell mediated gene therapy that contains non-transduced (hChonJ) and transduced (hChonJb#7) human allogeneic chondrocytes The hChonJb#7 cells were transduced with retrovirus encoding TGF-1 gene The study was a multicenter, randomized, single-blind, placebo-controlled phase IIb trial (NCT01671072) The OA patients (n = 54) were randomized into two groups; TG-C (n=27, 1.8x107cells, 3.5 ml/ knee) and placebo (n=27, normal saline, 3.5 ml/knee) TG-C or saline was injected directly to the knee joints and clinical evaluations were made at 12 and 24 weeks post treatment The primary evaluation endpoint was the changes of the International Knee Documentation Committee (IKDC) which measures pain, sports activities, and daily function Secondary evaluation endpoints were Western-Ontario and MacMaster University (WOMAC) score, Knee Injury and Osteoarthritis Outcome Score (KOOS), and 100 mm Visual Analogue Scale (VAS) Blood samples were analyzed to detect the replication competent retrovirus (at 12 and 24 weeks post treatment), TGF-1 DNA and protein (starting from 2 weeks up to 6 months post treatment) TG-C treatment showed signifi cant improvement in the primary and secondary clinical evaluations of IKDC (P <0.05) and

100 mm VAS (P <0.05) compared to the placebo as shown in table

1 Serum TGF-1 protein levels were within normal ranges (65ng/ ml) RCR and TGF-1 DNA were not detected at the completion of the study

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Molecular Therapy Volume 21, Supplement 1, May 2013 Copyright © The American Society of Gene & Cell Therapy

S218

DNA VECTOROLOGY & GENE TARGETING II

Table 1 Changes in Scores of the Primary and the Secondary Evaluation Parameters

at 24 Weeks Post Treatment

(N=27) P values primary endpoint IKDC 16.39±15.63 8.05±11.18 P=0.0304

WOMAC -13.81±19.23 -7.50±13.02 P=0.1692

Secondary endpoint KOOS -22.96±27.15 -13.92±16.85 P=0.1508

VAS -24.83±27.29 -10.79±18.22 P=0.0321

In summary, the current Phase Iib study indicated that TG-C treatment

improved the primary and the secondary clinical evaluation criteria

for pain, sports activities, and quality of daily life in patients with

knee OA when compared to the placebo control

566 A Novel Oncolytic HSV Design Based on

the Common Over-Expression of miR-21 in Tumors

Marco Marzulli,1 Lucia Mazzacurati,1 Bonnie Reinhart,1 Mark E

Hatley,2 Joseph C Glorioso,1 Paola Grandi,3 Justus B Cohen.1

1 Microbiology and Molecular Genetics, University of Pittsburgh,

Pittsburgh, PA; 2 Oncology, St Jude Children’s Research Hospital,

Memphis, TN; 3 Neurological Surgery, University of Pittsburgh,

Pittsburgh, PA.

Oncolytic virus (OV) therapy is aimed at the selective destruction

of cancer cells without harming healthy tissue Typically, OVs contain

mutations that block lytic virus replication in normal cells but that are

complemented in cancer cells OVs derived from herpes simplex virus

1 (oHSV) have shown effi cacy in preclinical models of several types

of cancer and safety in Phase I human trials, but therapeutic outcomes

have been disappointing This is due in part to the attenuating

mutations in these viruses that provide tumor selectivity, but also

reduce the viral lytic replication activity in tumors Recent studies

have demonstrated that lytic virus replication can be brought under

the control of cellular microRNAs (miRNAs) that are differentially

expressed in normal and cancer cells MiRNAs negatively regulate

gene expression by binding to complementary mRNA targets

MiRNA expression profi les of cancer cells typically show both

up-regulated and down-up-regulated miRNAs, but each cancer type has a

unique miRNA signature showing dysregulation of different sets of

miRNAs Thus targeted therapies based on these unique signatures

are applicable only to the corresponding cancer type Remarkably,

studies of a wide range of cancer types have identifi ed one miRNA,

the oncomir miR-21, which is up-regulated nearly universally in

cancer cells For example, increased miR-21 expression has been

reported in 27 types of cancer These results and functional studies

are consistent with a key role for miR-21 in the development and/

or maintenance of the neoplastic state (e.g., Chan et al., Cancer Res

65:6029-33, 2005; Hatley et al., Cancer Cell 18:282-93, 2010; Ma

et al., PNAS 108:10144-9, 2011) We have sought to take advantage

of the common up-regulation of miR-21 in cancer cells by creating

a novel control circuit in which the host cell miRNA induces, rather

than represses, lytic HSV replication To accomplish this, we have

engineered a miR-21-responsive dominant negative (dn) version of

the essential HSV replication gene, UL9, into the HSV genome The

recombinant virus (KGdn9T21) showed replication comparable to

that of a control virus without dnUL9 gene in cancer cell lines such as

U2OS, A431, A549, and in mouse embryonic fi brobasts (MEFs) In

contrast, KGdn9T21 replicated very poorly compared to the control

in the absence of miR-21, such as in Vero cells and miR-21-/- MEFs

These results support the potential of this vector design to serve as

a safe and effective oHSV platform that provides a general level of

safety and effi cacy for the treatment of cancer

567 Comparing the Activity of TALENs Constructed with Different Guanine-Targeting RVDs

Yanni Lin,1 Thomas J Cradick,1 Eli J Fine,1 Gang Bao.1

1 Biomedical Engineering, Georgia Institute of Technology and Emory University, Atlanta, GA.

The ability of designing the modular DNA-binding domains of TAL Effector Nucleases (TALENs) has led to their effective use in genomic editing in a number of biological applications The DNA-binding domain of TALENs contains a tandem array of 33-35 amino-acid repeats Each repeat specifi es one nucleotide through the 12th and 13th amino acids, named the repeat-variable di-residues (RVDs) The RVDs NI, HD and NG specifi cally bind to nucleotides A, C, and T respectfully in the target sequence However, multiple RVDs, such as NN (Asn-Asn), NK (Asn-Lys) and NH (Asn-His) recognize nucleotides G with different levels of specifi city and binding affi nity The NN RVD binds to either G or A with similar effi ciency; the

NK RVD has higher specifi city but lower affi nity in binding to G compared with NN Although according to previous studies of TAL effectors, the NH RVD might have high affi nity and high specifi city

in binding to G, it is still unclear if TALENs constructed with NH will have higher on-target activity than those constructed with RVDs

NN or NK Here we report the comparison of TALENs constructed using NK, NH, and NN (KNH TALENs) respectively that target six specifi c DNA cleavage sites To investigate how the number of Gs

in the target sequence correlates with the relative activities of the use

of each RVD, we compared individual TALEN monomer activity

of KNH TALENs using a single-strand annealing assay We further quantifi ed the off-target effects of KNH TALENs The results from this study will provide insight into the selection of G-specifying RVDs based on both cleavage activity and target specifi city, and thus help optimize TALEN designs for genome engineering

568 In Vivo Cleavage of Transgene Donors Promotes Nuclease-Mediated Targeted Integration

Sandra Cristea,1 Yevgeniy Freyvert,1 Yolanda Santiago,1 Michael

C Holmes,1 Fyodor D Urnov,1 Philip D Gregory,1 Gregory J Cost.1

1 Sangamo BioSciences, Richmond, CA.

Targeted DNA integration is commonly used to eliminate position effects on transgene expression Integration can be targeted to specifi c sites in the genome via both based and homology-independent processes Both pathways start the integration process with a site-specifi c break in the chromosome, typically from a zinc-fi nger nuclease (ZFN) We previously described an effi cient homology-independent targeted integration technique that captures short (<100 bp) pieces of DNA at chromosomal breaks created by ZFNs We show here that inclusion of a nuclease target site on the donor plasmid followed by in vivo nuclease cleavage of both the donor and the chromosome results in effi cient integration of large, transgene-sized DNA molecules into the chromosomal double-strand break Successful targeted integration via in vivo donor linearization

is demonstrated at fi ve distinct loci in two mammalian cell types, highlighting the generality of the approach Finally, we show that CHO cells, a cell type recalcitrant to homology-based integration, are profi cient at capture of in vivo-linearized transgene donors Moreover, we demonstrate knockout of the hamster FUT8 gene via the simultaneous ZFN- or TALE nuclease-mediated integration of

an antibody cassette Our results enable effi cient targeted transgene addition to cells and organisms that fare poorly with traditional homology-driven approaches

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