Article Figures & Data
Figures
- Figure 1 Identification of AGO-Abs
(A) Immunostaining of the adult rat brain with CSF (1:10) of patient XI resulted in a strong reactivity with the stratum pyramidale of the hippocampus, granule cells of the cerebellum, and cerebral cortex. The scale bar is indicated for each magnification. (B) Immunoprecipitation of the antigens with patient XI's CSF. Note the specifically enriched band around 100 kDa (*) and the less intense band around 280 kDa (**) obtained after immunoprecipitation with patient XI's CSF (E+) compared with control CSF (E−). Protein bands were visualized via silver staining. (C) Quantified reactivities of 12 patients with SNN, 22 patients with ONPs, and 9 HCs with 7 AGO variants on protein microarrays. Red dots represent significantly positive samples (z-score > 4). AGO-Abs = antibodies against the Argonaute protein family; HC = healthy control; ONP = other peripheral neuropathy; SNN = sensory neuronopathy.
- Figure 2 Confirmation of AGO-Abs Using Cell-Based Assay
HEK293 cells were transfected with VP5-AGO1-4 or VP5-TNRC6B plasmid, for a transient overexpression. Fixed and permeabilized cells were then immunostained with anti-HA antibody (in green) and patient XI's CSF (1:10; in red). Only AGO-transfected cells (in green) reacted with patient XI's CSF. No reactivity was observed on TNRC6B-transfected cells or nontransfected cells. Scale bar = 50 μm. AGO-Abs = antibodies against the Argonaute protein family.
- Figure 3 Neuroimaging Findings in 2 Patients With AGO2-Abs
(A) Axial (top) and coronal (bottom) fluid-attenuated inversion recovery (FLAIR) brain MRI of patient XII with limbic encephalitis, showing bilateral swelling and hyperintensity of medial temporal lobes, extending also to a lesser degree to the lateral temporal cortex. (B) Axial FLAIR (top) brain MRI of patient XVII presenting with cerebellar ataxia, showing vermis atrophy and left cerebellar hemisphere hyperintensity. Pancerebellar atrophy is better demonstrated on the sagittal T2 brain MRI (bottom) of the same patient.
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