The Patient Service Core brings to our patients, their families, community members, physicians and other health professionals, state-of-the-art educational, counseling, and diagnostic services made available because of the core of physician-scientists and allied health professionals associated with the Boston Comprehensive Sickle Cell Center. Through our outreach programs, we make services of the Boston Comprehensive Sickle Cell Center available to community members not receiving primary care at our major teaching hospitals. Professional counseling is provided to patients and families at risk for sickle cell disease and related disorders. We provide education to health professionals about sickle cell disease and related disorders. Education is also provided to patients and families at risk for sickle cell disease and related disorders. We also have established a hemoglobin diagnosis laboratoryproviding hemoglobinopathy screening using innovative technology and definitive hemoglobin diagnosis by mass spectrometry. A major limitation of HPLC for hemoglobinopathy detection is present during the study of sickle hemoglobinopathies where a clinically important interference exists between HbA2 and an adduct of HbS when using the Bio Rad Variant II method. Capillary zone electrophoresis, an alternative to isoelectric focusing, has found many uses in the research laboratory due to its simplicity, superior resolution, linearity, precision, reproducibility, speed of analysis, and automated operation. Using capillary zone electrophoresis the quantitation of HbA2 is not affected by the presence of HbS and capillary zone electrophoresis can resolve HbA1c from carbamylated HbA1a, A1b, A1d, F, C, and O-Arab. We will explore and develop the use of capillary zone electrophoresis for routine hemoglobinopathy and thalassemia detection.

Agency
National Institute of Health (NIH)
Institute
National Heart, Lung, and Blood Institute (NHLBI)
Type
Specialized Center--Cooperative Agreements (U54)
Project #
5U54HL070819-02
Application #
6900244
Study Section
Special Emphasis Panel (ZHL1)
Project Start
2004-05-20
Project End
2008-03-31
Budget Start
2004-05-20
Budget End
2005-03-31
Support Year
2
Fiscal Year
2004
Total Cost
$71,662
Indirect Cost
Name
Boston Medical Center
Department
Type
DUNS #
005492160
City
Boston
State
MA
Country
United States
Zip Code
02118
Maron, Bradley A; Oldham, William M; Chan, Stephen Y et al. (2014) Upregulation of steroidogenic acute regulatory protein by hypoxia stimulates aldosterone synthesis in pulmonary artery endothelial cells to promote pulmonary vascular fibrosis. Circulation 130:168-79
Cottrill, Katherine A; Chan, Stephen Y; Loscalzo, Joseph (2014) Hypoxamirs and mitochondrial metabolism. Antioxid Redox Signal 21:1189-201
Loscalzo, Joseph; Handy, Diane E (2014) Epigenetic modifications: basic mechanisms and role in cardiovascular disease (2013 Grover Conference series). Pulm Circ 4:169-74
Zhao, Yuzheng; Yang, Yi; Loscalzo, Joseph (2014) Real-time assessment of the metabolic profile of living cells with genetically encoded NADH sensors. Methods Enzymol 542:349-67
Barroso, Madalena; Florindo, Cristina; Kalwa, Hermann et al. (2014) Inhibition of cellular methyltransferases promotes endothelial cell activation by suppressing glutathione peroxidase 1 protein expression. J Biol Chem 289:15350-62
Joseph, Jacob; Loscalzo, Joseph (2013) Selenistasis: epistatic effects of selenium on cardiovascular phenotype. Nutrients 5:340-58
Maron, Bradley A; Tang, Shiow-Shih; Loscalzo, Joseph (2013) S-nitrosothiols and the S-nitrosoproteome of the cardiovascular system. Antioxid Redox Signal 18:270-87
Maron, Bradley A; Waxman, Aaron B; Opotowsky, Alexander R et al. (2013) Effectiveness of spironolactone plus ambrisentan for treatment of pulmonary arterial hypertension (from the [ARIES] study 1 and 2 trials). Am J Cardiol 112:720-5
Handy, Diane E; Loscalzo, Joseph; Leopold, Jane A (2013) Systems analysis of oxidant stress in the vasculature. IUBMB Life 65:911-20
Nallamshetty, Shriram; Chan, Stephen Y; Loscalzo, Joseph (2013) Hypoxia: a master regulator of microRNA biogenesis and activity. Free Radic Biol Med 64:20-30

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