Kelch-like Protein 11 (KLHL11) Antibodies in Children With Seizures of Undetermined Cause

JOHN TZARTOS; MARIA PECHLIVANIDOU; DESPOINA BOSVELI; ELPINICKIE NINOU; HANDE YUCEER; BEYZANUR YALCIN; CEM ISMAIL KUCUKALI; ERDEM TUZUN; SOCRATES TZARTOS; DILSAD TURKDOGAN

doi:10.21873/invivo.13445

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Figure 1.
Cell-based assay results. Transfected HEK293 cells expressing Kelch-like protein 11 (KLHL11) antigen were incubated with sera from (A) patients with paraneoplastic neurological disorders, (B) patients diagnosed with pediatric epilepsy of undetermined cause, (D) patients with myelin oligodendrocyte (MOG) antibody-associated disease and (E) healthy controls. Transfected cells were also incubated with commercial rabbit anti-KLHL11 IgG (red, C). Some patients with (A) paraneoplastic disorders and (B) with pediatric epilepsy were found positive for KLHL11 antibody at 1/1,000 dilution, whereas (D) the patient with MOG-antibody positive sera and (E) a healthy control gave no staining at 1/100 dilution. (F) HEK293 cells expressing glial fibrillary acidic protein (GFAP) did not immunoreact with patient sera or the commercial antibody. Scale bar: 20 μm. Note that the dot-like reactivity present in the GFAP-transfected HEK293 cells is considered as a background noise and is due to the non-specific proteins which are present in the human serum.
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Figure 2.
Co-localization studies with sera of patients and commercial Kelch-like protein 11 (KLHL11) antibody (Ab). (A, D) Reactivity (red) of sera of representative KLHL11 Ab positive patients with paraneoplastic neurological disorder (PNS) and pediatric epilepsy with HEK293 cells expressing KLHL11. (B, E) Reactivity (green) of a commercial KLHL11 Ab. (C, F) the commercial Ab shows significant co-localization with patients’ antibodies (yellow). Original magnification 40×.
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Figure 3.
Immunolabeling (brown color) of frozen rat brain sections with sera of Kelch-like protein 11 (KLHL11) antibody (Ab) positive and negative pediatric epilepsy (PE) patients and a healthy control. Sera of PE patients with KLHL11 Ab show cytoplasmic staining in neurons of deep cerebellar nuclei and brainstem (leftmost two columns), whereas no appreciable immunoreactivity is observed with sera of antibody negative PE patients and healthy control (rightmost two columns; original magnification 40×).