Identification of NPC phenotypes in a Npc1 inhibitor and npc1 morpholino zebrafish larvae model. NPC phenotypes were induced in the zebrafish larvae using either the Npc1 inhibitor, U18666A (2 µg/ml), or npc1 morpholino (npc1 MO) injected at the 1–2 cell stage with DMSO and Sham controls as necessary; a Morphology of 48 h post fertilisation (hpf) zebrafish by light microscopy. Quantitative behavioural analysis of 24 hpf zebrafish measuring number of spontaneous coils over 3 min in 2 µg/ml U18666A treated (b) and npc1 morphant (c) zebrafish, *p < 0.05, **p < 0.01, ****p < 0.0001, Kruskal–Wallis test with Dunn’s post-hoc correction. Representative images of LysoTracker Green staining of 96 hpf control and U18666A treated zebrafish larvae eyes with and without 500 µM miglustat co-treatment (d). Scale bars = 280 µm. N = 3–5, with a minimum of 10 fish per N

Identification of SLOS phenotypes in Dhcr7 inhibitor and dhcr7 morpholino zebrafish larvae models. SLOS phenotypes were induced in the zebrafish larvae using either Dhcr7 inhibitors AY9944 (75 µM) or trazodone (traz, 75 µM), or dhcr7 morpholino (dhcr7 MO) injected at the 1–2 cell stage with DMSO and Sham controls as necessary; a Morphology of 72 hpf zebrafish by light microscopy. Arrowheads indicate heart defects. Quantitative analysis of 72 hpf zebrafish by light microscopy; b eye area. Quantitative behavioural analysis of 72 hpf inhibitor treated zebrafish; c heart rate over 1 min and d escape response time. Quantitative behavioural analysis of 72 hpf dhcr7 morphant zebrafish; e heart rate over one minute and f escape response time. All analysis by Kruskal–Wallis test with Dunn’s post-hoc correction (b, c, d) or one-way ANOVA with Tukey post-hoc correction (e, f). *p < 0.05, **p < 0.01, ****p < 0.0001. Scale bars = 280 µm. N = 3, with a minimum of 3–10 fish per N

Cholesterol is reduced in SLOS model zebrafish and increased in NPC model zebrafish. Representative images of filipin staining of 96 hpf zebrafish to observe cholesterol distribution; a Filipin staining of SLOS zebrafish models induced using either the Dhcr7 inhibitor AY9944 (75 µM) or dhcr7 morpholino (dhcr7 MO) injected at the 1–2 cell stage. Images of the zebrafish head and upper body from a lateral view (left) or dorsal view (right); b Filipin staining of NPC zebrafish models induced using either npc1 morpholino (npc1 MO) injected at the 1–2 cell stage or the Npc1 inhibitor, U18666A (2 µg/ml). Images of the zebrafish head from a dorsal viewpoint. Brain areas are indicated by coloured bars above images. Scale bars = 280 µm. N = 3, with a minimum of ten fish per N

Ganglioside GM1 is increased in both SLOS and NPC zebrafish models. Representative images of cholera toxin subunit B (CtxB) staining of 96 hpf zebrafish to observe ganglioside GM1 distribution; a CtxB staining of SLOS zebrafish models induced using the Dhcr7 inhibitors AY9944 (75 µM) or trazodone (traz) (75 µM). Images of the zebrafish head from a lateral view (left) or dorsal view (right); b CtxB staining of NPC zebrafish models induced using either npc1 morpholino (npc1 MO) injected at the 1–2 cell stage or the Npc1 inhibitor, U18666A (2 µg/ml). Images of the zebrafish head from a dorsal viewpoint. Brain areas are indicated by coloured bars above images. Please note that the NPC images were taken using a separate Z.1 light sheet microscope that had been set up slightly differently. Scale bars = 280 µm. N = 3, with a minimum of ten fish per N

ZFIN is incorporating published figure images and captions as part of an ongoing project. Figures from some publications have not yet been curated, or are not available for display because of copyright restrictions.