| Stedman
H, Browning K, Oliver N, Oronzi-Scott M, Fischbeck
K, Sarkar S, Sylvester J, Schmickel R, Wang K. Nebulin cDNAs
detect a 25 kilobase transcript in skeletal muscle and localize
to human chromosome two. Genomics 2(1):1-7, Jan. 1988.
Oronzi-Scott M, Sylvester JE, Heiman-Patterson T, Shi Y-J,
Fieles W, Stedman H, Burghes A, Ray P, Worton
R, Fischbeck KH. Duchenne muscular dystrophy gene expression
in normal and diseased human muscle. Science 239(4846):1418-1420,
Mar. 18, 1988.
Cooper BJ, Winand NJ, Stedman HH, Valentine
BA, Hoffman EP, Kunkel LM, Oronzi-Scott M, Fischbeck K, Kornegay
J, Williams JR, Sylvester JE. The homologue of the Duchenne
locus is defective in X-linked muscular dystrophy of dogs.
Nature 334(6178):154-156, July 14, 1988.
Eller M, Stedman HH, Sylvester JE, Fertels
SH, Rubinstein NA, Kelly AM, Sarkar S. Nucleotide sequence
of full length human embryonic myosin heavy chain cDNA. Nuc
Acids Res 17(9):3591-3592, May 11, 1989.
Stedman H, Sylvester J, Pette D, Giger U.
Establishment of canine M-type PFK deficiency as a mo-del
for the study of somatic cell gene replacement therapy. J
Cellular Biochemistry 13B:174, 1989.
Eller M, Stedman HH, Sylvester JE, Fertels
SH, Rubinstein NA, Kelly AM, Sarkar S. Human em-bryonic myosin
heavy chain cDNA: Interspecies sequence conservation of the
myosin rod, chromo-somal locus and isoform specific transcription
of the gene. FEBS Lett 256(1-2):21-28, Oct. 9, 1989.
Stedman HH, Eller M, Jullian EH, Fertels
SH, Sarkar S, Sylvester JE, Kelly AM, Rubinstein NA. The human
embryonic myosin heavy chain: Complete structure reveals evolutionary
relationships with other developmental isoforms. J Biol Chem
265(6):3568-3576, Feb. 25, 1990.
Stedman HH, Kelly AM, Rubinstein NA. Isoform-specific
cDNAs for human embryonic, neonatal, and slow skeletal myosin
heavy chains. Ann NY Acad Sci 599:119-126, 1990.
Stedman HH, Sweeney HL, Shrager JB, Maguire
HC, Panettierri RA, Narusawa M, Leferovich JM, Sladky JT,
Kelly AM. The mdx mouse diaphragm reproduces the degenerative
changes of Duchenne muscular dystrophy. Nature 352(6335):536-539,
Aug. 8, 1991.
Shrager JB, Naji A, Kelly AM, Stedman HH.
A PCR-based assay for the wild-type dystrophin gene transferred
into the mdx mouse. Muscle & Nerve 15(10):1133-1137, Oct.
1992.
Giger U, Smith BF, Woods CB, Patterson DF, Stedman
H. Inherited phosphofructokinase defi-ciency in an
American cocker spaniel. J Am Vet Med Assoc 201(10):1569-1571,
Nov. 15, 1992.
Petrof BJ, Shrager JB, Stedman HH, Kelly
AM, Sweeney HL. Dystrophin protects the sarcolemma from stresses
developed during muscle contraction. Proc Natl Acad Sci USA
90(8):3710-3714, Apr. 15, 1993.
Petrof BJ, Stedman HH, Shrager JB, Eby J,
Sweeney HL, Kelly AM. Adaptations in myosin heavy chain expression
and contractile function in the mdx mouse diaphragm. Am J
Physiol 265(3 Pt 1): 834-841, Sept. 1993.
Sarkar S, Stedman H, Du Y, Prakash, KJ,
Zheng ZC. The cytoplasmic translation inhibitory RNA species
of chick embryonic muscle: Correlation of biological activity
with secondary structure. Biochem (Life Sci. Adv.) 12:109-120,
1993.
Stedman HH, Carpenter JP, Shlansky-Goldberg
RD. (1994) Percutaneous balloon catheter vascular control
for infected axillary artery pseudoaneurysm. J Cardiovasc
Surg 35(6):529-531, Dec. 1994.
Jullian EH, Kelly AM, Hoffman R, Pompidou AJ, Schiaffino
S, Stedman HH, Rubinstein NA. Characterization
of a human perinatal myosin heavy-chain transcript. European
J Biochem 230(3):1001-1006, June 15, 1995.
Smith BF, Henthorn PS, Rajpurohit Y, Stedman H,
Wolfe JH, Patterson DF, Giger U. A cDNA encoding muscle-type
phosphofructokinase. Gene 168(2):275-276, Feb. 12, 1996.
Wang K, Knipfer M, Huang QQ, van Heerden A, Hsu LC, Gutierre
G, Quian XL, Stedman H. Human skeletal muscle
nebulin sequence encodes a blueprint for thin filament architecture:
Sequence motifs and affinity profiles of tandem repeats and
terminal SH3. J Biol Chem 271(8):4304-4314, Feb. 23, 1996.
Smith BF, Stedman HH, Rajpurohit Y, Henthorn
PS, Wolfe JH, Patterson DF, Giger U. Molecular basis of canine
muscle-type phosphofructokinase deficiency. J Biol Chem 271(33):
20070-20074, Aug. 16, 1996.
Ehlen-Haecker S, Stedman HH, Balice-Gordon
RJ, Smith DBJ, Greelish JP, Mitchell MA, Wells A, Sweeney
HL, Wilson JM. In vivo expression of full length dystrophin
from adenoviral vectors deleted of all viral genes. Hum Gene
Ther 7(15):1907-1914, Oct. 1, 1996.
Chowrashi PK, Pemrick SM, Li S, Yi P, Clarke T, Maguire B,
Ader G, Saintigny P, Mittal B, Tewari M, Stoeckert C, Stedman
HH, Sylvester JE, Pepe FA. The myosin filament XV
assembly: Contribu-tions of 195 residue segments of the myosin
rod and the eight C-terminal residues. J Musc Res & Cell
Motil 17(5):555-573, Oct. 1996.
Greelish JP, Su LT, Lankford EB, Burkman, JM, Chen H, Konig
SK, Mercier IM, Desjardins PR, Mitchell MA, Zheng XG, Leferovich
J, Gao GP, Balice-Gordon RJ, Wilson JM, Stedman HH.
Stable restoration of the sarcoglycan complex in dystrophic
muscle perfused with histamine and a recombinant adeno-associated
viral vector. Nature Medicine 5(4):439-443, Apr. 1999. (Cover
Photo)
Stedman H, Mendell J, Wilson JM, Finkle
R, Kleckner AL. Phase I clinical trial utilizing gene therapy
for limb girdle muscular dystrophy: Alpha, beta, gamma or
delta-sarcoglycan gene delivered with intramuscular instillations
of adeno-associated vectors. Hum Gene Ther 11:777-790, Mar.
20, 2000.
Shrager JB, Desjardins PR, Burkman JM, Konig SK, Stewart
DR, Su L, Shar MC, Tewari M, Hoffman R, Rickels MR, Jullian
EH, Rubinstein NA, Stedman HH. Human skeletal
myosin heavy chain genes are tightly linked in the order embryonic-IIa-IId/x-IIb-perinatal-extracocular.
J Musc Res Cell Motility 21(4):345-355, May 2000.
Shrager JB, Kim DK, Hashmi YJ, Lankford EB, Wahl P, Stedman
HH, Levine S, Kaiser LR. Lung volume reduction surgery
restores the normal diaphragmatic length-tension relationship
in emphysematous rats. J Thorac Cardiovasc Surg. 21(2):217-24,
Feb. 1, 2001.
Shrager JB, Kim DK, Hashmi YJ, Stedman HH,
Kaiser LR, Levine S. Sarcomeres are added in series to emphasematous
rat diaphragm. Chest 121 (3): 831-5 Mar. 2002.
Dejardins P, Burkman J, Shrager J, Allmond L, Stedman
H. Evolutionary implications of three novel members
of the human sarcomaric myosin heavy chain gene family. Mol.
Biol. Evol. 19 (4): 375-93 Apr. 2002.
Bridges CR, Burkman JM, Malekan R, Konig SK, Chen H, Yarnell
CB, Gardner TJ, Stewart AS, Stecker M, Patterson T, Stedman
HH. Global cardiac-specific transgene expression
using cardiopulmonary bypass with cardiac isolation. Ann.
Thor. Surg. 73(6):1939-1946, June 2002.
Konig S, Burkman J, Fitzgerald J, Mitchell M, Su L, Stedman
H. Modular organization of phylogenetically conserved
domains controlling developmental regulation of the human
skeletal myosin heavy chain gene family. J. Biol. Chem. 277(31):27593-27605,
Aug. 2, 2002.
Krupnick AS, Zhu J, Nguyen T, Kreisel1 D, Balsara1 KR, Lankford
EB, Clark CC, Levine S, Stedman HH, Shrager
JB. Inspiratory loading does not accelerate dystrophy in the
mdx mouse diaphragm: Implications for regenerative therapy.
J. Appl. Phys. 2003 (in press).
Abstracts
Lesniewski J, Su L, Burkman J, Wang Z, Zheng X, Bridges C,
Stedman H. A small animal model for isolated
cardiac perfusion and recirculation in situ. Selected for
Presentation at 5th Annual Meeting, ASGT, Boston, MA, June
2002.
Su L, Lesniewski J, Burkman J, Wang Z, Zheng X, Bridges C,
Stedman H. Development of a small animal model for
extracorporeal circulatory support during endothelial permeabilization
for systemic gene therapy. Selected for Presentation at 5th
Annual Meeting, ASGT, Boston, MA, June 2002.
Su L, Lesniewski J, Burkman J, Wang Z, Zheng X, Bridges C,
Stedman H. Development of a small animal
model of circulatory isolation to promote efficient vector
access to skeletal muscle in the pelvic girdle and lower limbs.
Selected for Presentation at 5th Annual Meeting, ASGT, Boston,
MA, June 2002.
Konig S, Burkman J, Stedman H. Identification
of evolutionarily conserved promoter sequences responsible
for powerful muscle. Selected for Presentation at 5th Annual
Meeting, ASGT, Boston, MA, June 2002.
Konig S, Burkman J, Stedman H. Critical
role of putative MyoD and MEF-2 binding sites in the construction
of a powerful MYH 3-based muscle-specific transcriptional
cassette. Selected for Presentation at 5th Annual Meeting,
ASGT, Boston, MA, June 2002.
Arruda VR, Stedman H, Nichols T, Haskins
M, Schuettrummpf J, Herzog RW, Liu YL, Wagner K, Couto L,
High KA. Sustained correction of hemophilia B phenotype following
intravascular delivery of AAV vector to skeletal muscle. Selected
for Presentation at 5th Annual Meeting, ASGT, Boston, MA,
June 2002.
Konig S, Burkman J, Stedman H. Experimental
verification of bioinformatic predictions using the MYH family
of muscle-specific promoters. Selected for Presentation at
the Tenth International Congress on Neuromuscular Diseases.
Konig S, Burkman J, Stedman H. Three New
Candidate Genes for Dominantly Heritable Sarcomeric Myosinopathy.
Selected for Presentation at the Tenth International Congress
on Neuromuscular Diseases.
Stedman HH, Kozyak BW, Nelson A, Thesier
DM, Shrager JB, Bridges CR, Minugh-Purvis N, Mitchell MA.
Inactivating mutation in the MYH 16 superfast myosin gene
abruptly reduced the size of the jaw closing muscles in a
recent human ancestor. Selected for presentation at the 6th
Annual Meeting, ASGT, Washington, DC, June 2003. Full manuscript
with identical authorship currently in review at Nature.
Editorials, Reviews, Chapters and Participation
in Committee Reports
Dasgupta S, Eller MS, Zolnay S, Jayabaskaran C, Stedman
H, Sarkar S. Cytoplasmic translation inhibitory RNA
of chick embryonic muscle: possible role in myogenesis as
antimes¬senger RNA. In Emerson C, Fischman D, Nadal-Ginard
B and Siddiqui M (eds). Molecular Biology of Muscle Develop¬ment/
UCLA Symposium on Molecular and Cellular Biology, Vol. 29.
New York: Alan R. Liss, Inc, 1986, 591-603.
Stedman H, Sarkar S. Molecular genetics
in basic myology: a rapidly evolving perspective. [Review]
Muscle & Nerve 11(7):668-693, July 1988.
Stedman H, Sarkar S. Molecular genetics
in muscular dystrophy research: revolutionary progress. [Review]
Muscle & Nerve 11(7):694-704, July 1988.
Stedman H, Browning K, Qian X, Oliver N,
Oronzi-Scott M, Fischbeck K, Sarkar S, Sylvester J, Schmickel
R, Wang K. Characterization of the human nebulin locus and
its 25 kb transcript: progress towards a full length cDNA.
In Stockdale F and L Kedes (eds): Cellular and Molecular Biology
of Muscle Development/ UCLA Symposia on Molecular and Cellular
Biology, Vol. 93. New York: Alan R. Liss, Inc., 1989, 181-196.
Sarkar S, Eller MS, Raychowdhury MK, Stedman H,
Wu QL. The cytoplasmic translation inhibitory RNA of chick
embryonic muscle: resolution of multiple biologically active
subspecies and mechanism of action. In Stockdale F and L Kedes
(eds): Cellular and Molecular Biology of Muscle Development/
UCLA Symposia on Molecular and Cellular Biology, Vol. 93.
New York: Alan R. Liss, Inc., 1989, 555-570.
Shrager JB, Stedman HH, Sweeney HL, Petrof
B, Kelly AM. The mdx mouse and the mdx diaphragm: Implications
for the pathogenesis of Duchenne muscular dystrophy. In Kelly,
AM and HM Blau (eds): Neuromuscular Development and Disease.
New York: Raven Press, 1992, 317-328.
Molecular approaches to therapy for Duchenne and limb-girdle
muscular dystrophy. Curr. Opin. Mol. Ther. 3 (4): 350-6, Aug.
1, 2001.
Alternative Media
CD-ROM and DVD Digital Photographs and Movies: Case Studies
in Clinical Surgery
Multimedia Tool for Interactive Use in Academic Surgery
1) Clinical Teaching in General and Vascular Surgery
2) Complex Surgical Procedures in the Research Laboratory
Although this rapidly evolving resource exists in a form
that complicates its representation in a typewritten curriculum
vitae, an overall description is warranted. When implemented
on a portable computer with an ultra-fast hard drive, the
catalog and digital media database to be described provide
instantaneous access to a wide-reaching compendium of basic
and clinical teaching material. The immediate relevance of
the “basic science” and “teaching case”
material to our patient care mission has strengthened the
integration of these all too often competing features of the
academic surgical landscape.
On the previous recommendation of Clyde F. Barker, M.D.,
I have been archiving video, audio, and high-resolution still
photographic media files relating to a wide range of topics
in academic surgery. In my laboratory, teaching files on complex
microvascular surgical procedures were initially used as interactive,
self-paced tools to accelerate learning while standardizing
the sequence of events in critical experiments. The audio-visual
files proved especially helpful in our more recently developed
cardiac surgical procedures in large animal models. Current
advances in computer hardware and software have dramatically
accelerated the processes of media acquisition, transfer,
and recovery, allowing a transition from lecture format to
interactive use in a wide range of venues. All of the files
have been reformatted for representation in keyword searchable
database catalogs to ensure instantaneous availability. For
portable use, over 1000 Photographs and compressed video files
currently fit onto a single DVD-ROM disk.
For many VA patients, the database now includes a chronological
depiction of preoperative studies, operative procedure (including
audio and video of key technical details), gross pathology
in situ and ex vivo, histopathology, and perioperative clinical
information (SICU monitoring parameters, lab values). In the
hospital, we have been using the combination of still and
video image reproduction to enhance the learning environment
for medical students and residents while optimizing the continuity
of care for our patients. Although portions of the overall
resource are being developed for use over the internet, the
full capability of is best appreciated with the system running
in the classroom or conference. This novel resource is being
rapidly incorporated into our programs at the Philadelphia
Veterans Affairs Medical Center, with plans to expand this
month to the general teaching exercises of the Department
of Surgery using the William T. Fitts Surgical Education Center.
In the latter venue I envision its full integration with the
existing teaching tools in use under the direction of G. Paul
Dabrowski , M.D., Director, Medical Student Education.
Patents
U.S. Patent 6,177,403 B1 [H. Stedman] "Compositions,
Methods and Apparatus for Delivery of a Macromolecular Assembly
to an Extravascular Tissue of an Animal." Issued Jan.
23, 2001 44 claims.
International Patent Application PCT/US99/04367 (WO 99/43360)
[H. Stedman & J. Wilson] "Stable
Protection from Dystrophic Sarcoleminal Degeneration and Restoration
of the Sarcoglycan Complex."
International Patent Application PCT/US98/27072 (WO 99/31982)
[H. Stedman & C. Bridges] "Transvascular
Delivery of a Composition to an Extravascular Tissue of a
Mammal."
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