FIGURE

Fig. 6

ID

ZDB-FIG-221023-29

Publication

Marom et al., 2021 - COPB2 loss of function causes a coatopathy with osteoporosis and developmental delay

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Fig. 6

Figure 6. Secretion of collagen type II in copb2^b1327/b1327 zebrafish mutants and ER/Golgi defects in Copb2^+/− mouse fibroblasts can be rescued by treatment with ascorbic acid

(A–F) Confocal sections of zebrafish notochords (anterior is to the left and dorsal to the top, images were taken at the level of the yolk extension at 30 hpf). Immunolabeling of type II collagen in untreated copb2 siblings (A) and copb2^b1327/b1327 (B) embryos, 100 mM ascorbic acid-treated copb2 siblings (C) and copb2^b1327/b1327 embryos (D), and 200 mM ascorbic acid-treated copb2 siblings (E) and copb2^b1327/b1327 embryos (F). See Figure S9 for quantification of the distribution of phenotypes in zebrafish.

(G) Representative image of Copb2^+/− mouse fibroblasts (Het) and wild-type littermate control cells (WT) labeled by CellLight Golgi-GFP reagent for visualization of Golgi structure.

(H) Graph summarizing multiple single-cell measurements of Golgi area with or without ascorbic acid (n = 1,631/1,723 Het cells −/+ ascorbic acid treatment and n = 1,021/1,152 WT cells −/+ treatment).

(I) BiP/mHspa5 expression in Copb2^+/− mouse fibroblasts (Het, n = 3) and wild-type littermate control cells (WT, n = 2) with or without ascorbic acid. Results are presented as mean values ± SD, summary of three independent experiments. ^∗∗p = 0.002.

Expression Data

Antibody:	Ab1-col2a
Fish:	AB/TU copb2^b1327/b1327 (AB/TU)
Condition:	chemical treatment by environment: ascorbic acid
Anatomical Term:	notochord
Stage:	Prim-15

Expression Detail

Antibody Labeling

Phenotype Data

Fish:	copb2^b1327/b1327 (AB/TU)
Condition:	chemical treatment by environment: ascorbic acid
Observed In:	notochord
Stage:	Prim-15

Phenotype Detail

Acknowledgments

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