Re: Pathology 28
nrp - 11-04-06 23:36

D.Spectrin.The disease is hereditory spherocytosis.Rt?But isnt it that the manifestations of HS wont b seen during early years of life?

pathology
Radonco - 11-05-06 01:08

spectrin-dis. is hereditory spherocytosis.

Re: Pathology 28
Youngdoctor - 11-05-06 23:05


spectrin-dis. is hereditory spherocytosis.


Is spectrin is protein?.Please explain to me.

Re: Pathology 28
TheOne - 11-06-06 11:17

Explanation: The correct answer is D. The infant is suffering from hereditary spherocytosis, a disease that can result from defective erythrocyte cytoskeletal proteins such as spectrin or ankyrin. Spectrin is a cytoskeletal protein in the erythrocyte that binds to the ankyrin/band 3 protein complex. It also binds to a protein 4.1/actin complex to form a stable supporting cytoskeletal network just below the plasma membrane. This helps to maintain the biconcave shape of the cell. Defective spectrin can lead to a destabilization of this network and a tendency of the affected cells to assume a spherical shape and become osmotically fragile. Such cells often become trapped in the splenic cords, and are subsequently destroyed by phagocytes. This can lead to hemolytic anemia, jaundice and splenomegaly. Clathrin (choice A) is a protein found on the cytoplasmic side of the plasma membrane. It is located within the coating of the so-called coated pits seen in electron micrographs of cells engaged in receptor-mediated endocytosis. Connexon (choice B) is a protein found in gap junctions, usually aggregated around the aqueous pores within the junctional area. Dynein (choice C) is an ATPase protein responsible for the interaction and sliding of microtubule doublets, resulting in the bending of cilia and flagella. Tubulin (choice E) is a protein that exists in alpha and beta forms that polymerize to form microtubules.

Re: Pathology 28
galaxy - 05-28-10 02:11

An infant born to German-American parents presents with mild anemia, jaundice, and splenomegaly. A complete blood count with differential reveals spherocytosis; the reticulocyte count is elevated. The parents state that several relatives have also suffered from a similar illness. The infant's condition is most likely caused by defective :

D. spectrin
hereditary spherocytosis is due to spectrin, ankyrin defects

Re: Pathology 28
babbu5508 - 06-21-10 15:20

D. spectrin deficiency hereditary spherocytosis.In a peripheral blood smear, the abnormally small red blood cells lacking the central pallor are seen

Re: Pathology 28
bingousmle - 06-30-10 22:06

Defective spectrin can lead to a destabilization of this network and a tendency of the affected cells to assume a spherical shape and become osmotically fragile.