Figure 8.

Figure 8. Abnormal ER-Golgi trafficking of procollagen contributes to reduced collagen in the extracellular matrix in Klhl40a deficiency.

(A) Immunofluorescence of control and klhl40a KO zebrafish muscle (3 months). Mutant muscle displays the intracellular accumulation of procollagen (as seen with co-labeling with sarcomeric α-actinin; white arrow) in klhl40a KO muscle compared to +/+control. Mutant muscle showed reduced levels of collagen compared to controls (white arrow). Integrinβ1 level is also reduced in the ECM in the mutant muscle. (B) Retention using selective hooks (RUSH) assay for ER-Golgi trafficking of procollagens and (C) sialyltransferase (ST) in control and KLHL40 knockout human myoblasts. The Golgi apparatus is marked with a yellow dashed line. N=33–45 cells in each group; data are mean ± S.E.M; and Tukey’s HSD test (p<0.01).