FIGURE

Fig. 4

ID

ZDB-FIG-251104-13

Publication

Mero et al., 2025 - HPDL Biallelic Variants in Cerebral Palsy and Childhood-Onset Hereditary Spastic Paraplegia: Human and Zebrafish Insights

Other Figures

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Fig. 4

Drug tests. (A) Analysis of reactive oxygen species (ROS) production in two 4-hydroxyphenylpyruvate dioxygenase-like protein (HPDL) patients' fibroblasts after 1 week of treatment with 4-hydroxybenzoic acid (4HB) 1 mM and 4-hydroxymandelic acid (4HMA) 5 mM. Data showed a significant effect of 4HMA on ROS production, while no effects of 4HB treatment were observed. Data are the averages of two experiments. (B) 4HB 1 μM treatment improved burst activity of hpdl-F0 embryos versus controls; 4HMA 5 μM treatment normalized the phenotype of hpdl-F0 embryos (controls, n = 114; controls+4HB, n = 115; controls+4HMA, n = 51 hpdl-F0, n = 85; hpdl-F0+4HB, n = 74; hpdl-F0+4HMA, n = 42). (C) Locomotor behavior (distance moved and velocity) of hpdl-F0 versus controls at 5 dpf (days post-fertilization) was rescued with 4HMA 5 μM treatment, but not with 4HB 1 μM treatment (controls, n = 107; controls+4HB, n = 114; controls+4HMA, n = 111 hpdl-F0, n = 69; hpdl-F0+4HB, n = 57; hpdl-F0+4HMA, n = 44). In all graphs, asterisks denote statistically significant differences: *P ≤ 0.05, **P ≤ 0.01, ***P ≤ 0.001, ****P ≤ 0.0001, whereas “ns” indicates non-significant results.

Expression Data

Expression Detail

Antibody Labeling

Phenotype Data

Fish:	AB AB + CRISPR1-hpdl
Conditions:	chemical treatment by environment: 4-hydroxybenzoic acid chemical treatment by environment: 4-hydroxymandelic acid
Knockdown Reagent:	CRISPR1-hpdl
Observed In:	locomotion swimming behavior
Stage Range:	Prim-15 to Day 5

Phenotype Detail

Acknowledgments

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