Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0153640 (Cerebellum)
 1,777 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Cerebellum development depends on the correct differentiation of progenitors into neurons, a process controlled by a transcriptional program that remains poorly understood. Here we show that neural-specific deletion of the BTB/POZ zinc-finger transcription factor-encoding gene Rp58 (Znf238, Zfp238) causes severe cerebellar hypoplasia and developmental failure of Purkinje neurons, Bergmann glia and granule neurons. Deletion of Rp58 in mouse embryonic Atoh1(+) progenitors leads to strong defects in growth and foliation owing to its crucial role in the differentiation of granule neurons. Analysis of the Rp58 mutant at E14.5 demonstrates that Rp58 is required for the development of both glutamatergic and GABAergic neurons. Rp58 mutants show decreased proliferation of glutamatergic progenitors at E14.5. In addition, Rp58 ablation results in a reduced number of GABAergic Pax2(+) neurons at E16.5 together with defects in the transcriptional program of ventricular zone progenitors. Our results indicate that Rp58 is essential for the growth and organization of the cerebellum and regulates the development of both GABAergic and glutamatergic neurons.
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PMID:Rp58 is essential for the growth and patterning of the cerebellum and for glutamatergic and GABAergic neuron development. 2251 77

In the human cerebellum, the crus I and crus II lobules (or the ansiform lobule), which are implicated in cognitive and visuomotor functions, are significantly expanded compared to other anterior and posterior lobules, which are involved mainly in somatosensorimotor function. In applying rodent models, it is essential to identify the lobules that are homologous to human crus I and crus II. Observation of the lobular structure in human, macaque, marmoset, rat, and mouse has indicated that human crus I and II are homologous to crus I in rodents (referred to as "ansiform area, AA"). This new lobular definition is supported by lobule-based mapping of the olivocerebellar climbing fiber and Purkinje cell (PC) projection patterns in rodents; crus II and simple lobules are innervated by the mediocaudal part of each inferior olive subnucleus and project to the dorsal part of the cerebellar nuclei, while crus I (or the AA) is innervated by the rostrolateral part of each inferior olive subnucleus and projects to the ventral part of the cerebellar nuclei. Concerning zebrin stripes, the central lobules (lobules VI-VII and AA or crus I in rodents) show a laterally expanded arrangement solely of positive stripes. Our recent analysis has shown that this arrangement of zebrin-positive stripes in the AA originates from their developmental process. Between E14.5 and E17.5, lateral protrusion and shift has been observed in the domains of protocadherin 10-positive PC subsets (which would become zebrin-positive later) in the central area of the immature cerebellum that eventually becomes lobules VI-VII and AA or crus I. These data suggest that the AA (or crus I in rodents) is a unique lobule in the mammalian cerebellum which is characterized by distinct connectivity from neighboring lobules, a massive expansion in skillful primates, and the formation of longitudinal stripes different from that in neighboring anterior and posterior lobules.
Cerebellum 2018 Feb
PMID:Crus I in the Rodent Cerebellum: Its Homology to Crus I and II in the Primate Cerebellum and Its Anatomical Uniqueness Among Neighboring Lobules. 2928 17