Chorein Expression In Brain Regions

In non-affected control brains we found chorein expressed at approximately the same level in all examined brain regions (Fig. 5). A similar pattern was found also in substantia nigra, globus pallidus, occipital cortex and cerebellum (data not shown). Full length chorein is represented by a band at approx. 370 kDa (consistent with the predicted molecular weight of chorein of 360 kDa). This band was absent in tissue from ChAc cases (Fig. 5).

In control brain tissue, we found at least two clear bands at approximately 160 kDa and 94 kDa that were not present in ChAc tissue. The origin of these bands is unclear. They represent proteins of distinct size matching chorein epitopes within the amino acids 27-326 of full length chorein due to the synthetic peptide used for the antibody production. This comprises approximately the first 10% of chorein's N-terminus [5]. It is unlikely that they result from unspecific degradation products since these bands do not vary in size and are present in all examined regions. These proteins may represent N-terminal fragments of 25% (94 kDa) and 43% (160 kDa), respectively, of full length chorein e.g. due to alternative splicing or clear defined post-translational modifications.

Bands at approximately 310 kDa and 82 kDa were present in brain tissue from ChAc patients as well as from controls, and therefore correspond most likely to nonspecific cross reactions of the polyclonal chorein antiserum used for detection in Western blot.

Fig. 4 Stereological data show an increased proportion of glial cells within the striatum in ChAc patients. (a) Cell numbers of astroglial and oligodendroglial cells (grey) and neurons (black) estimated [12] for the striatum of two ChAc cases, the mean of five HD patients and the mean of five non-affected controls. (b) The striatal glial index was calculated

Fig. 4 Stereological data show an increased proportion of glial cells within the striatum in ChAc patients. (a) Cell numbers of astroglial and oligodendroglial cells (grey) and neurons (black) estimated [12] for the striatum of two ChAc cases, the mean of five HD patients and the mean of five non-affected controls. (b) The striatal glial index was calculated

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