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Figure 4

ID

ZDB-IMAGE-231204-38

Antibodies

Ab2-pink1

Publication

Serrano et al., 2023 - Genetic model of UBA5 deficiency highlights the involvement of both peripheral and central nervous systems and identifies widespread mitochondrial abnormalities

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Figure 4

Ultrastructural analysis of the peripheral nerves and skeletal muscle in uba5^ex1s/ex1s. (A) Patterning of the peripheral nerves and skeletal muscle at 6 dpf (i–vi). Electron microscopy images show mitochondrial abnormalities, including elongated mitochondria (ii) and degenerating mitochondria (iii), in the nerve terminals of uba5^ex1s/ex1s fish (arrowhead). Degradation vacuoles enclosing debris (vi), and autophagic structures associated with mitochondria in the skeletal muscle (v), are also observed. (B) Patterning of the skeletal muscle at 14 dpf shows abnormal mitochondria with swollen cristae (ii) and vacuolated mitochondria (iii) in uba5 mutants (arrowhead). (C) Western blot for Pink1 and Direct Blue staining (loading control) on protein lysates obtained from pooled samples of 25 embryos at 6 dpf. M, mitochondria; NT, nerve terminal; PN, peripheral nerve; SV, synaptic vesicles.

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