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Article

Intracellular degradation of histidine-rich glycoprotein mutants: tokushima-1 and 2 mutants are degraded by different proteolytic systems.

Journal of biochemistry - 1 Aug 2000

Wakabayashi S, Yoshida H, Shigekiyo T, Koide T

Abstract excerpt

We reported the first case of a congenital histidine-rich glycoprotein deficiency (HRG Tokushima) in which substitution of Gly85 with Glu (G85E) in the first cystatin domain resulted in intracellular degradation and a low plasma level of HRG [Shigekiyo, T. et al. (1998) Blood 91, 128-133]. Recently, we identified the gene mutation of a second case of HRG deficiency as a Cys223 to Arg (C223R) mutation in the...

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PMID: 10920255
DOI: 10.1093/oxfordjournals.jbchem.a022742

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HRG

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