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PMID: 426037 Published · ppublish English Journal Article Research Support, U.S. Gov't, P.H.S.

alpha-Actinin deficiency in thrombasthenia: possible identity of alpha-actinin and glycoprotein III.

The American journal of pathology ·Vol. 94 ·No. 3 ·1979-03-00 ·Pages 509-28

Gerrard JM, Schollmeyer JV, Phillips DR, White JG

Abstract

Blood platelets contain a variety of contractile protein species, including the glycoprotein alpha-actinin, which is found at the Z disc in skeletal muscle cells. In the present study, we have considered the possibility that alpha-actinin might be one of several previously described platelet surface glycoproteins. Purified anti-alpha-actinin antibody was found to react strongly with partially purified platelet glycoprotein III, weakly with platelet glycoprotein IIb, and not at all with platelet glycoproteins Ib and IV. Platelets from three siblings with thrombasthenia, a disorder characterized by severe deficiency of platelet glycoproteins IIb and III, were found also to be equally deficient in alpha-actinin. These findings indicate that alpha-actinin and glycoprotein III are identical and suggest that this protein may be an anchor point for actin on the inside of the membrane. Combined with ultrastructural studies of normal and thrombasthenic platelets, the new findings provide a clearer understanding of contraction in single cells and small aggregates.

MeSH Terms
Actinin/blood,deficiency,immunology Blood Platelet Disorders/blood,pathology Blood Platelets/analysis,ultrastructure Blood Proteins/analysis Cross Reactions Glycoproteins/blood,immunology Humans Membrane Proteins/blood,immunology Muscle Proteins/deficiency Platelet Adhesiveness
Chemicals
Blood Proteins Glycoproteins Membrane Proteins Muscle Proteins Actinin
Authors & Affiliations
4 authors, click to expand affiliations / ORCID
Gerrard J M
Schollmeyer J V
Phillips D R
White J G
References (35)
35 references, click to expand
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Article Info
Journal
The American journal of pathology
Abbr.
Am J Pathol
ISSN
0002-9440
Published
1979-03-00
Pages
509-28
Language
English
Region
United States
NLM ID
0370502
PMCID
PMC2042274
Subset
IM
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