Amy L. Ellenbogen

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BACKGROUND Factors which influence lesion size from catheter-based cryoablation have not been well described. This study describes factors which influence lesion size during catheter cryoablation. METHODS AND RESULTS Cryoablation was delivered to porcine left ventricular myocardium in a saline bath using 4- or 8-mm electrode catheters. Ablation was(More)
Inherited deficiency of the enzyme adenosine deaminase (ADA) results in a syndrome of severe combined immunodeficiency (SCID). Children with ADA- -SCID lack ADA in all cells and tissues. In contrast, a "partial" deficiency of ADA has been described in six immunologically normal children from four different "families." These children lack ADA in their(More)
Complete genetic deficiency of adenosine deaminase (ADA) results in a fatal syndrome of severe combined immunodeficiency (SCID). Genetic partial deficiency of ADA, with no detectable enzyme activity in erythrocytes but with variable amounts of enzyme activity detectable in other cells, is usually associated with normal immunologic function but can give rise(More)
Lysosomal acid alpha-glucosidase (EC 3.2.1.3) hydrolyzes 1,4-linked alpha-D-glucose polymers present in glycogen. Genetic deficiency of acid alpha-glucosidase results in glycogen-storage disease type II, encompassing a spectrum of disorders of varying severity. To study the molecular basis for this heterogeneity, we sought to clone the coding sequence for(More)
BACKGROUND Irrigated radiofrequency (RF) ablation catheters may produce different lesion sizes dependent upon the electrode orientation to the tissue. This study examined the effect of irrigated electrode orientation on the lesion size and explores a potential mechanism for this effect. METHODS AND RESULTS Lesions were created in isolated porcine(More)
OBJECTIVE Creation of transmural myocardial lesions with epicardial surgical devices to treat atrial fibrillation is difficult. A new cooled bipolar radiofrequency ablation probe was used to create transmural myocardial lesions under controlled conditions. METHODS The Coolrail (AtriCure, Inc, West Chester, Ohio) is a handheld probe with 2 parallel 30-mm(More)
We have further regionally localized the gene for human acid alpha glucosidase (GAA) to 17q21→q23 by examinaiton of hybrid clones derived from a fusion between human fibroblasts carrying a 17/19 balanced translocation (17pter→17q23::19p13.3→19pter; 19qter→p13.3::17q23→17qter) and a mouse line deficient in thymidine kinase. These hybrids were constantly(More)
We have identified and/or characterized at least nine RFLPs at the adenosine deaminase (ADA) locus, detected by digestion of DNA with MspI, BanII, PstI, BalI, and PvuII. The RFLPs were distributed over approximately 15 kb of the gene, from IVS 2 to IVS 10. They exhibited Mendelian inheritance and were in Hardy-Weinberg equilibrium. For seven fully(More)