Figure 3.
Immunoblotting of platelet lysates revealed severely reduced levels of GPIbα and glycosylated β1 integrin. Platelet lysates of controls, healthy relatives, and patients from both pedigrees were immunoblotted with anti-GPIbα, anti-β1 integrin, anti-β3 integrin, anti-TPO receptor, anti-VWF, and anti-β-actin as an internal control. Band intensities in blots were quantified by densitometric analysis using the ‘Image J’ software. Bar graphs represent each protein expression relative to β-actin. Impaired GPIbα and β1 integrin levels were observed in the proband from both pedigrees. VWF level was also reduced in pedigree A patients. VWF, von Willebrand factor.

Immunoblotting of platelet lysates revealed severely reduced levels of GPIbα and glycosylated β1 integrin. Platelet lysates of controls, healthy relatives, and patients from both pedigrees were immunoblotted with anti-GPIbα, anti-β1 integrin, anti-β3 integrin, anti-TPO receptor, anti-VWF, and anti-β-actin as an internal control. Band intensities in blots were quantified by densitometric analysis using the ‘Image J’ software. Bar graphs represent each protein expression relative to β-actin. Impaired GPIbα and β1 integrin levels were observed in the proband from both pedigrees. VWF level was also reduced in pedigree A patients. VWF, von Willebrand factor.

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