ACE phenotyping in Gaucher disease.

ABSTRACT:

Background

Gaucher disease is characterized by the activation of splenic and hepatic macrophages, accompanied by dramatically increased levels of angiotensin-converting enzyme (ACE). To evaluate the source of the elevated blood ACE, we performed complete ACE phenotyping using blood, spleen and liver samples from patients with Gaucher disease and controls.

Methods

ACE phenotyping included 1) immunohistochemical staining for ACE; 2) measuring ACE activity with two substrates (HHL and ZPHL); 3) calculating the ratio of the rates of substrate hydrolysis (ZPHL/HHL ratio); 4) assessing the conformational fingerprint of ACE by evaluating the pattern of binding of monoclonal antibodies to 16 different ACE epitopes.

Results

We show that in patients with Gaucher disease, the dr

SUBMITTER: Danilov SM 

PROVIDER: S-EPMC5891352 | biostudies-literature | 2018 Apr

REPOSITORIES: biostudies-literature