We have analyzed the expression pattern of protocadherin-19, a member of the delta2-protocadherins, in the nervous system of developing zebrafish using in situ hybridization methods. mRNA encoding protocadherin-19 (Pcdh19) began to be expressed at about 12 hours post fertilization (hpf) showing a segmental expression pattern in the anterior 1/3 of the neural keel, with strong expression in the presumptive forebrain, cerebellum/rhombomere 1 and rhombomere 4. Pcdh19 expression in the posterior neural keel was continuous and confined to the midline region. By 24 hpf, Pcdh19 was expressed widely in the brain and spinal cord, with higher expression levels in the ventral telencephalon, dorsal and central thalamus, optic tectum, central tegmentum, cerebellum and dorsolateral regions of the hindbrain. As development proceeded, Pcdh19 expression domains became restricted to the dorsal and/or lateral regions of the central nervous system, and Pcdh19 expression was not detected in the spinal cord of two- and three-day old embryos. Pcdh19 was also expressed by the eye primordium, developing retina, lens and otic vesicle. Similar to its expression in the brain, Pcdh19 expression in the eye and ear was also spatially and temporally regulated.
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http://dx.doi.org/10.1387/ijdb.092882ql | DOI Listing |
Pediatr Neonatol
August 2024
Division of Pediatric Neurology, Chang Gung Children's Hospital and Chang Gung Memorial Hospital, Taoyuan, Taiwan; Chang Gung University College of Medicine, Taoyuan, Taiwan. Electronic address:
Mol Brain
August 2024
Department of Anatomy, Graduate School of Medical Science, Brain Korea 21 Project, Yonsei University College of Medicine, Seoul, 03722, Republic of Korea.
Protocadherin 19 (Pcdh19) is a homophilic cell adhesion molecule and is involved in a variety of neuronal functions. Here, we tested whether Pcdh19 has a regulatory role in axon guidance using the developing Xenopus retinotectal system. We performed targeted microinjections of a translation blocking antisense morpholino oligonucleotide to knock down the expression of Pcdh19 selectively in the central nervous system.
View Article and Find Full Text PDFFront Cell Neurosci
April 2024
Department of Neurology, University of Michigan, Ann Arbor, MI, United States.
Open Biol
April 2024
School of Biomedicine and Robinson Research Institute, University of Adelaide, Adelaide, South Australia 5005, Australia.
Non-clustered protocadherins (ncPcdhs) are adhesive molecules with spatio-temporally regulated overlapping expression in the developing nervous system. Although their unique role in neurogenesis has been widely studied, their combinatorial role in brain physiology and pathology is poorly understood. Using probabilistic cell typing by sequencing, we demonstrate combinatorial inter- and intra-familial expression of ncPcdhs in the developing mouse cortex and hippocampus, at single-cell resolution.
View Article and Find Full Text PDFEpilepsy Behav
May 2024
Child Neurology Division and Children's Epilepsy Monitoring Unit, Children's Medical Centre, Tehran University of Medical Sciences, Tehran, Iran.
PCDH19 clustering epilepsy (PCDH19-CE) is an X-linked epilepsy disorder associated with intellectual disability (ID) and behavioral disturbances, which is caused by PCDH19 gene variants. PCDH19 pathogenic variant leads to epilepsy in heterozygous females, not in hemizygous males and the inheritance pattern is unusual. The hypothesis of cellular interference was described as a key pathogenic mechanism.
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