Lisa Ho & Praise Austin Dr. Robin W. Briehl Albert Einstein - - PowerPoint PPT Presentation

Lisa Ho & Praise Austin

• Dr. Robin W. Briehl

Albert Einstein College of Medicine

 Red Blood Cell

 Round disc shape  Transports oxygen

 Sickle Cell Trait  Sickle Cell Disease

 Abnormal sickle shape  Rigidity  Cell Damage & Cell-Cell Adhesion  Hemolysis & Anemia

 Glutamic Acid  Valine  Deoxygenation  Hydrophobic Interaction  Polymerization into Stiff Fibers  Distorts shape & rigidifies cells  Induces Vaso-Occlusion

 Video camera  Zeiss light microscope  DIC Optics  Epi-illumination light source for

photolysis of HbCO

 Potassium Phosphate buffer (pH 7.2

at 0.1 M concentration)

 Blood or Hb solution sample  Gel sample

 Fiber Visualization using 546nm light source  Differential Interference Contrast Microscopy  Can visualize objects smaller than diffraction

limitation size

 Splits the light into two different light beams  Wollaston prism  The phase image facilitates visualization of

the fibers.

VCR Image Processor Video Camera Barrier Filter Analyzer 546nm

• bservation

436nm photolysis Dichroic mirror Hg arc Field Diaphragm Exciter Filter CO Hg arc Wollaston prism Slide Wollaston prism Polarizer

 Help decipher how polymers grow

and dissolve

 Aim:

 Inhibit growth  Facilitate dissolution

 Increase the delay time  Conduct Rheological tests on Lab

grown Gels

 Clinical Studies on various therapeutic

treatments



Enbury, S. H., Hebbel, R. P., Mohandas, N. & Steinberg, M. H. (1994). Sickle cell disease: basic principles and clinical practice. New York: Raven Press Ltd.



Dickerson, R. E. & Geis, I. (1983). Hemoglobin. California: The Benjamin/ Cummings Plublishing Company, Inc.



Wang, J. C., Turner, M. S., Agarwal, G., Kwong, S., Josephs, R., Ferrone, F.

• A. & Breihl, R. W. (2002). Micromechanics of isolated sickle cell

hemoglobin fibers: bending moduli and persistence lengths. Journal of Molecular Biology, 315, 601-612.



Agarwal, G., Wang, J. C., Kwong, S., Cohen, S. M., Ferrone, F. A., Josephs, R. & Breihl, R. W. Sickle hemoglobin fibers: mechanisms of

• depolymerization. Journal of Molecular Biology, 322, 395-412.



Ferrone, F. A. (2004). Polymerization and sickle cell disease: a molecular

• view. Microcirculation, 11:2, 115-128



Breihl, R. W. (1997). Sickle cell hemoglobin. Encyclopedia of human biology, (Second Edition, Vol. 8, pp. 1-20). New York, Academic Press Inc.



Anfinsen, C. B., Edsall, J. T., Richards, F. M. & Eisenberg, D. S. (1987). Advances in protein chemistry. New York, Academic Press Inc.

• Professor Robin W. Briehl, MD
• Jiang Cheng Wang
• Suzanna Kwong
• Albert Einstein College of Medicine & Staff
• Dr. Sat Bhattacharya
• Harlem Children Society
• The audience

Any questions?