[Band 3 deficiency as a cause of hereditary spherocytosis].
Rinsho Ketsueki
; 56(7): 837-45, 2015 Jul.
Article
en Ja
| MEDLINE
| ID: mdl-26251147
ABSTRACT
Band 3 protein accounts for the largest percentage of whole erythrocyte membrane proteins. Abnormalities in this protein are closely associated with pathologies including hereditary spherocytosis (HS), Southeast Asian ovalocytosis and distant renal tubular acidosis. Currently, EMA binding capacity measurement in erythrocytes is the most useful screening test for diagnosing HS. We have also demonstrated reduced EMA binding capacity in patients with HS who have deficiencies of membrane proteins such as ankyrin not directly binding to EMA and who have as yet undetectable abnormalities of membrane proteins. However, even patients with hereditary elliptocytosis, who have a partial spectrin deficiency, were found to show reduced EMA binding capacity. Six of 7 had spherocytic elliptocytosis. Therefore, it is necessary to meticulously diagnose HS by ruling out all other possibilities.
Texto completo:
1
Colección:
01-internacional
Banco de datos:
MEDLINE
Asunto principal:
Esferocitosis Hereditaria
/
Proteína 1 de Intercambio de Anión de Eritrocito
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Ancirinas
/
Membrana Eritrocítica
Límite:
Humans
Idioma:
Ja
Revista:
Rinsho Ketsueki
Año:
2015
Tipo del documento:
Article