Figure 4.

Increased levels of CMA components in lysosomes from HD cells. A, Immunoblots for the indicated proteins of MEFs from ¹⁸QHtt and ¹¹¹QHtt knock-in mice maintained in medium supplemented (Serum+) or not (Serum−) with serum. Right, Quantification of the changes in LAMP-2A and LAMP-1 relative to their values in ¹⁸QHtt serum+. Values are mean ± SE, n = 5. B, Immunoblots for the indicated proteins of homogenates (Hom), cytosol (Cyt), and lysosomes with high CMA activity (Lys) isolated from livers of ¹⁸QHtt and ¹¹¹QHtt knock-in mice. Right, Quantification of the changes in the indicated proteins in the lysosomal fraction relative to their values in ¹⁸QHtt lysosomes. Values are mean ± SE n = 4. C, Immunoblots for LAMP-2A and hsc70 of total lysosomes (Lys total), lysosomal membranes (Lys mb), and lysosomal matrices (Lys mtx) isolated from the same animals. Right, Percentage of lysosomal LAMP-2A present at the lysosomal membrane (mb) and matrix (mtx) in each group of lysosomes. Differences with control are significant for *p < 0.05.