Hyperactive bone marrow

A photograph of Dr. Max Perutz s two angstrom-per-centi-meter x-ray diffraction model of hemoglobin is shown in Plate 3. Hemoglobin, the blood pigment that carries oxygen and makes the blood red, Is composed of four chains, two alpha globln chains 

ACS Symposium Series American Chemical Society Washington, DC, 1980. 

This Inability to make the beta globln chains In Cooley s anemia, which consequently means an Inability to make hemoglobin, together with the precipitation of uncomplexed alpha globln chains causing all the red cells made to be lysed, accounts for the signs and symptoms seen In the disease Cooley s anemia. 

The only treatment for this disease Is blood transfusion, but with every unit of blood, as we said before, several hundred mg of Iron are deposited In the body and this deposition of Iron results In severe symptoms and ultimately In death. The body Is normally equipped to handle about one mg of Iron a day In the diet. What happens to the organs of patients with this massive deposition of excess Iron  

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Lighter fuels, benzene, toluene, cleaning fluids (carbon tetrachloride), petrol, paraffin, and even the fluorocarbon propellants found in various household sprays and medications have all been used, particularly by children, to produce changes in consciousness. They are all inhaled, often with the aid of a plastic bag, and, since they are lipid-soluble, they are readily concentrated in brain tissue. As with many anesthetics there is an early period of hyperactivity, excitement, and intoxication, followed by sedation and confusion. Prolonged or regular use can cause serious toxicity, with bone-marrow depression, cardiac dysrhythmias, peripheral neuropathy, cerebral damage, and liver and kidney disorders (1). 

Fig. 15.3 The fusion protein has the annino-ternninal part of the Ber kinase and the carboxy-ternninal part of the Abl tyrosine kinase. The result is that the Abl kinase in the fused protein is hyperactive and drives proliferation of haematopoietic cells in the bone marrow. (See also Rgs 24-25 of ref. 31, reproduced with permission of Taylor and Francis, Inc.)...

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