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Published Erratum
. 2021 Sep 8;11(1):18218.
doi: 10.1038/s41598-021-97068-y.

Publisher Correction: Age-dependent ataxia and neurodegeneration caused by an αII spectrin mutation with impaired regulation of its calpain sensitivity

Arkadiusz Miazek  1   2 , Michał Zalas  1 , Joanna Skrzymowska  1 , Bryan A Bogin  3 , Krzysztof Grzymajło  2 , Tomasz M Goszczynski  1 , Zachary A Levine  3   4 , Jon S Morrow  5   6 , Michael C Stankewich  7
Affiliations
Published Erratum

Publisher Correction: Age-dependent ataxia and neurodegeneration caused by an αII spectrin mutation with impaired regulation of its calpain sensitivity

Arkadiusz Miazek et al. Sci Rep. .
No abstract available

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Figures

Figure 1
Figure 1
Relationship of the R1098Q variant to αII spectrin structure. (a) Schematic representation of αII spectrin with 20 complete homologous repeats each ≈ 106 residues and the location of selected functional domains that interrupt the repeat motif. The 9th and 10th repeats (red and purple respectively) harbor an inserted SH3 domain, a variably appearing alternative transcript insertion, and specialized sequences encompassing the site of CaM binding and the site Y1176 and G1177 that is preferentially cleaved by calpain. (b) Sequence of repeats 8–11 aligned by homology. The boxes denote the boundaries of each of the three α-helices (A–C) of the canonical repeat unit; these are separated by AB and BC loops and inter-unit linkers. The sequences representing the SH3 domain and the alternative transcript inserted into repeat 9 and the calpain and CaM interaction sites in repeat 10 are indicated. The arrow marks the site of the R1098Q mutation near the start of the 10th repeat. The YG site of preferred calpain cleavage is colored green. (c) Clustal alignment of residues flanking the R1098Q mutation. This locus, and the flanking region, is almost perfectly conserved across diverse species. Residues fully conserved are denoted with asterisks (*); residues strongly conserved with a score greater than 0.5 on the PAM250 matrix are designated with a colon (:). Weakly similar residues are denoted with a period (.).
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