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A new case of homozygous C1-inhibitor deficiency suggests a role for Arg378 in the control of kinin pathway activation  Alberto López-Lera, MS, Bertrand.

Published byJadwiga Wiśniewska Modified over 5 years ago

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Presentation on theme: "A new case of homozygous C1-inhibitor deficiency suggests a role for Arg378 in the control of kinin pathway activation  Alberto López-Lera, MS, Bertrand."— Presentation transcript:

1 A new case of homozygous C1-inhibitor deficiency suggests a role for Arg378 in the control of kinin pathway activation  Alberto López-Lera, MS, Bertrand Favier, PhD, Rocío Mena de la Cruz, MS, Sofía Garrido, BS, Christian Drouet, PhD, Margarita López- Trascasa, PhD  Journal of Allergy and Clinical Immunology  Volume 126, Issue 6, Pages e3 (December 2010) DOI: /j.jaci Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions

2 Fig 1 Biochemical and functional studies on the R378C mutant: Western blot of fresh plasma samples of the family members showing the native (105 kd) and cleaved/latent (96 kd) forms of C1-Inh. Equal plasma volumes were loaded in each lane. Normal human plasma (NHP) and purified C1-Inh (Berinert P) are shown as reference (A). C1-Inh’s interaction with C1s protease and kallikrein was examined by means of ELISA of Cos-7 cell supernatants (20 ng of protein per lane) and plasma samples (B). Journal of Allergy and Clinical Immunology  , e3DOI: ( /j.jaci ) Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions

3 Complement profile and family tree
Complement profile and family tree. The reference values were obtained from a series of healthy donors. The first generation was not available for the study. ND, Not detectable. Journal of Allergy and Clinical Immunology  , e3DOI: ( /j.jaci ) Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions

4 Follow-up of complement profile after treatment with stanozolol
Follow-up of complement profile after treatment with stanozolol. C1-Inh levels and function and C4 and C2 levels were markedly increased in plasma of the R378C homozygote after treatment with stanozolol. C1q increased to normal levels only in the second posttreatment sample 28 months after the beginning of stanozolol intake. All the measures are expressed as a percentage of the reference values in each case. Journal of Allergy and Clinical Immunology  , e3DOI: ( /j.jaci ) Copyright © 2010 American Academy of Allergy, Asthma & Immunology Terms and Conditions

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