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. 2023 Mar;270(3):1776-1780.
doi: 10.1007/s00415-022-11417-z. Epub 2022 Nov 4.

Rho GTPase-activating protein 17 (ARHGAP17) as additional autoimmune target in ARHGAP26-IgG/anti-Ca autoantibody-associated autoimmune encephalitis

Sven Jarius  1 , Jürgen Haas  2 , Brigitte Wildemann  2
Affiliations

Rho GTPase-activating protein 17 (ARHGAP17) as additional autoimmune target in ARHGAP26-IgG/anti-Ca autoantibody-associated autoimmune encephalitis

Sven Jarius et al. J Neurol. 2023 Mar.
No abstract available

Keywords: Autoantibody; Autoimmune encephalitis; Cerebellar ataxia; Cognitive decline; GTPase Regulator Associated with Focal Adhesion Kinase (GRAF); GTPase-activating protein 2 (ARHGAP2, N-chimerin, chimerin-1); Immunoglobulin G (IgG); Limbic encephalitis; Nadrin; Oligophrenin-like protein 1 (OPHN1L); Polyneuropathy; Rho GTPase-activating protein 10 (ARHGAP10, GRAF2); Rho GTPase-activating protein 17 (ARHGAP17); Rho GTPase-activating protein 26 (ARHGAP26); RhoGAP interacting with CIP4 homologs protein 1 (RICH-1).

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Conflict of interest statement

S.J., J.H. and B.W. report no conflicts of interest.

Figures

Fig. 1
Fig. 1
ARGAP17 expression in the human brain and sequence alignments of ARHGAP17, ARHGA26 and ARHGAP10 revealing a potential shared binding site. A ARHGAP17 expression levels in various subregions of the adult brain and cerebellum as well as in selected neuronal and glial cell types (modified images from the Human Protein Atlas image database [23]; https://www.proteinatlas.org; licensed under the Creative Commons Attribution-ShareAlike 3.0 International License). B Significant sequence homology of ARHGAP26, ARHGAP10 and ARHGAP17 within the RhoGAP domain (ARHGAP26: aa 383–568, ARHGAP10: aa 389–574, ARHGAP17: 252–442 aa) as detected using UniProt sequence data and ClustalO multiple sequence alignment tool (see Supplementary figure for additional information) [18, 24]. By contrast, no such significant sequence homology with ARHGAP26, ARHGA10 and ARHGAP17 in the RhoGAP domain exists in 12 other ARHGAPs studied in the same microarray experiment, all of which did not show significant binding of patient IgG (not shown); and homology only with the ALK_Y sequence of ARHGAP26, ARHGAP10 and ARHGAP17 (and the GALK_Y sequence of ARHGAP17) exists in the case of ARHGAP2 (N-chimerin), which yielded a weak signal in the same experiment (not shown). Together, this suggests that the RhoGAP domain could be the main binding site of the patient’s anti-ARHGAP IgG antibodies. Note that 2 (out of 7) isoforms of ARHGAP17 (Q68EM7-4 und Q68EM7-7) lack the region of interest
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References

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