|
|
 Previous Article | Table of Contents | Next Article 
A lower molecular weight gamma-chain variant in a congenital abnormal
fibrinogen (Kyoto)
N Yoshida, M Okuma, M Moroi and M Matsuda
A gamma-chain variant with a lower molecular weight than the normal gamma
chain was detected in a new congenital abnormal fibrinogen with impaired
polymerization of the fibrin monomer and with normal release of
fibrinopeptides A and B in a 45-year-old male. Purified fibrinogen analyzed
on SDS-polyacrylamide gel electrophoresis under the reduced condition
contained an abnormal protein band with an apparent molecular weight of
48,000 compared with the gamma chain with a molecular weight of 50,000.
This abnormal protein band was found to be a gamma-chain variant from the
molar ratio of A alpha chain:B beta chain:gamma chain:abnormal protein
(about 2:2:1:1), with positive staining for carbohydrate and crosslinking
ability. Crosslinked fibrin contained three types of gamma-gamma dimers
with apparent molecular weights of 94,000 (the same as normal major
gamma-gamma dimer), 92,000 and 90,000, and the plasmic digests of
crosslinked fibrin in the presence of calcium retained three types of
gamma-gamma dimer remnants. This suggests that the abnormal gamma-chain
variant has a shorter polypeptide chain not in the NH2-terminal but in the
COOH-terminal portion, probably at or near the polymerization site. This
patient's two daughters had the same abnormal fibrinogen. This unique
inherited abnormal fibrinogen was designated as fibrinogen Kyoto, and the
gamma- chain variant as gamma Kyoto.
Volume 68,
Issue 3,
pp. 703-707,
09/01/1986
Copyright © 1986 by The American Society of Hematology
 CiteULike  Connotea  Del.icio.us  Digg  Reddit  Technorati What's this?
This article has been cited by other articles:
|
|
|
|
 
J. L. Mullin, S. O. Brennan, P. S. Ganly, and P. M. George
Fibrinogen Hillsborough: a novel gamma Gly309Asp dysfibrinogen with impaired clotting
Blood,
May 15, 2002;
99(10):
3597 - 3601.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
T. Sugo, C. Nakamikawa, H. Takano, J. Mimuro, S.-i. Yamaguchi, M. W. Mosesson, D. A. Meh, J. P. DiOrio, N. Takahashi, H. Takahashi, et al.
Fibrinogen Niigata With Impaired Fibrin Assembly: An Inherited Dysfibrinogen With a Bbeta Asn-160 to Ser Substitution Associated With Extra Glycosylation at Bbeta Asn-158
Blood,
December 1, 1999;
94(11):
3806 - 3813.
[Abstract]
[Full Text]
[PDF]
|
|
|
|
|
|
|
H. C.F. Cote, S. T. Lord, and K. P. Pratt
gamma -Chain Dysfibrinogenemias: Molecular Structure-Function Relationships of Naturally Occurring Mutations in the gamma Chain of Human Fibrinogen
Blood,
October 1, 1998;
92(7):
2195 - 2212.
[Full Text]
[PDF]
|
|
|
|
|
