FIGURE

Fig. 5

ID

ZDB-FIG-170929-6

Publication

Gioia et al., 2017 - The chaperone activity of 4PBA ameliorates the skeletal phenotype of Chihuahua, a zebrafish model for dominant osteogenesis imperfecta

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Fig. 5

4PBA treatment ameliorated the tail shape (A) and normalized the neural spine slope (B) of Chi/+ fish after 14 weeks of treatment. Scale bar: 1 mm in (A) and 500 µm in (B). (C) Transmission electron microscopy of 11 dpf Chi/+ larvae showed a general reduction of the ER cisternae size after 4PBA treatment. Magnification 12000X. (D) Representative images of picro sirius red staining Chi/+ zebrafish regrown tail after 7 days from tail clipping in absence or presence of 4PBA. A strongest signal in the sample obtained from 4PBA treated fish confirmed a higher amount of collagen in the matrix following 4PBA treatment. White lines indicate the portion of tail regrown in 7 days. Magnification 10X.

Expression Data

Expression Detail

Antibody Labeling

Phenotype Data

Fish:	col1a1a^dc124/+
Condition:	chemical treatment by environment: sodium phenylbutyrate
Observed In:	osteoblast endoplasmic reticulum lumen
Stage:	Days 7-13

Phenotype Detail

Acknowledgments

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