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Mutations in the human Notch ligand jagged 1 (JAG1) result in a multi-system disorder called Alagille syndrome (AGS). AGS is chiefly characterized by a paucity of intrahepatic bile ducts (IHBD), but also includes cardiac, ocular, skeletal, craniofacial and renal defects. The disease penetration and severity of the affected organs can vary significantly and(More)
In nontransformed DHFR/G-8 cells (NIH 3T3 cells transfected with normal rat neu gene), the normal neu gene product was initially synthesized as a 170-kDa protein bearing endoglycosidase H-sensitive oligosaccharide chains and was then processed to a 175-kDa mature form with endoglycosidase H-resistant, endoglycosidase F-sensitive oligosaccharide chains. Most(More)
β-thalassemia major is a disorder of globin synthesis, resulting in anemia and compensatory bone marrow hyperproliferation. Conventional imaging studies do not measure bone marrow activity reliably. We report on the use of technetium-99m sestamibi (MIBI) in a β-thalassemia major patient treated with an allogeneic bone marrow transplant. Pre-transplant and(More)
Skin necrosis is a rare complication of heparin therapy. Strong evidence suggests an immune-mediated mechanism in which heparin-antibody complexes bind to platelets, resulting in platelet aggregation, thromboembolism, and ischemic necrosis. Heparin-induced thrombocytopenia (HIT) may also occur in response to immune-mediated platelet aggregation. The(More)
The large conductance voltage- and Ca(2+)-activated K(+) channel (MaxiK, BK(Ca), BK) is composed of four pore-forming α-subunits and can be associated with regulatory β-subunits. One of the functional roles of MaxiK is to regulate vascular tone. We recently found that the MaxiK channel from coronary smooth muscle is trans-inhibited by activation of the(More)
Using acridine orange as a reporter compound, we demonstrate that suramin enters and accumulates in low pH intracellular compartments (endosomes, lysosomes, and trans-Golgi complex) of normal and v-sis-transformed NIH 3T3 cells. The concentration of suramin in these acidic compartments is estimated to be > 150 microM, higher than the concentration known to(More)
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