The following protein defects can cause hereditiary spherocytosis except –
Correct Answer: Glycophorin C
Description: Hereditary spherocytosis is an inherited disorder caused by intrinsic defects in the RBC cell membrane skeleton that render red cells spheroid less deformable and vulnerable to splenic sequestration and destruction. Cell membrane contains chief protein component spectrin consists of alpha and beta polypeptide chain which form intervined heterodimers.The head region self associate to form tetramer while tail associate with actin oligomers. Spectrin actin skeleton is connected to cell membrane by two distinct interactions . 1 - proteins ankyrin and band 4.2 binds spectrin to transmembrane ion transpoer band 3. 2- protein 4.1 binds the tail of spectrin to another transmembrane protein glycophorin A. Palladin is a component of actin Glycophorin C deficiency is associated with HEREDITARY ELLIPTOCYTOSIS.GPC deficient RBCs are also paially deficient in 4.1R speculated to be the basis for the elliptocytosis Reference :1-pathologic basis of diseases by Robbins and Cotran.South Asia Edition volume 1.page no.632,633 2-Wintrobe's clinical hematology 13th edition .pages-707,709-712,720 Ref Robbins 9/e pg 410
Category:
Pathology
Get More
Subject Mock Tests
Practice with over 200,000 questions from various medical subjects and improve your knowledge.
Attempt a mock test nowMock Exam
Take an exam with 100 random questions selected from all subjects to test your knowledge.
Coming SoonGet More
Subject Mock Tests
Try practicing mock tests with over 200,000 questions from various medical subjects.
Attempt a mock test now