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Figure 2 | Orphanet Journal of Rare Diseases

Figure 2

From: Leucine-sensitive hyperinsulinaemic hypoglycaemia in patients with loss of function mutations in 3-Hydroxyacyl-CoA Dehydrogenase

Figure 2

GDH enzyme activity in control and patient lymphoblasts (a) Activity was measured, either without GTP or with increasing concentrations of GTP (0 – 1000nM) to determine the IC50. Activities are reported +/− SEM. (b.1.) Western blot of 50ug whole lymphoblast lysates (Lane 1 patient, Lane 2 control) or 300ug mitochondrial lymphoblast lysates immunoprecipitated with 1ug GDH rabbit polyclonal antibody (Lane 3 patient, Lanes 4–6 controls) then probed with mouse monoclonal antibody to HADH. (White lines indicated unused lanes cut out, all samples on same gel) (b.2.) Western blot of 300 μg mitochondrial lymphoblast lysates immunoprecipitated with 1 μg GDH rabbit polyclonal antibody (Lane 1 control, Lanes 2–4 patient samples) then probed with mouse monoclonal antibody to HADH.

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Orphanet Journal of Rare Diseases

ISSN: 1750-1172