2020
DOI: 10.1002/cbf.3582
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The role of Shh signalling pathway in central nervous system development and related diseases

Abstract: Sonic hedgehog (Shh) plays important roles in developmental of vertebrate animal central nervous system (CNS), and Gli is its downstream signal molecule. Shh signalling is essential for pattern formation, cell‐fate specification, axon guidance, proliferation, survival and differentiation of neurons in CNS development. The abnormal signalling pathway of Shh leads to the occurrence of many nervous system diseases. The mechanism of Shh signalling is complex and remains incompletely understood. Nevertheless, studi… Show more

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Cited by 52 publications
(38 citation statements)
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References 110 publications
(118 reference statements)
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“…Of those, five genes may be of particular interest because they are highly expressed in the brain and are also nominally associated with brain volumes. Zinc finger protein 521 (encoded by ZNF521), a protein acting as transcriptional factor, is involved in the sonic hedgehog pathway; this pathway has an essential role in the central nervous system development (Li et al, 2020;Matsubara et al, 2009;Scicchitano et al, 2019).…”
Section: Discussionmentioning
confidence: 99%
“…Of those, five genes may be of particular interest because they are highly expressed in the brain and are also nominally associated with brain volumes. Zinc finger protein 521 (encoded by ZNF521), a protein acting as transcriptional factor, is involved in the sonic hedgehog pathway; this pathway has an essential role in the central nervous system development (Li et al, 2020;Matsubara et al, 2009;Scicchitano et al, 2019).…”
Section: Discussionmentioning
confidence: 99%
“…SLOS is characterized by elevated 7dehydrocholesterol and low plasma cholesterol levels. Some of the major consequences of cholesterol deficiency include abnormal formation of the CNS, face and limbs via an effect on sonic hedgehog (SHH) signaling pathways during development (Li et al, 2020) and its effect on steroidogenesis, particularly that of dihydrotestosterone. Therapeutic intervention with supplementation of cholesterol and symptomatic treatment after early diagnosis decreases mortality and improves longterm outcome (Donoghue et al, 2018).…”
Section: Smith-lemli-opitz Syndromementioning
confidence: 99%
“…Throughout vertebrates’ CNS development, Shh is required and acts as a morphogenic factor in proliferation, differentiation, and survival of neural precursor cells [ 68 ]. In the brain, Shh protein is first expressed ventrally at the early development stages [ 69 , 70 ].…”
Section: Lncrnas and Pathways Involved In Non-syndromic Intellectumentioning
confidence: 99%
“…In the brain, altered regulations of the Shh signaling can lead to an extensive range of neurological disorders, brain tumors, and intellectual disability [ 73 , 74 ]. Variants in the Shh gene have also been found in patients showing development abnormalities, and speech and learning delays [ 70 , 75 ]. Several non-coding RNAs have been reported as having a role of regulation of the Shh pathway, including lncRNAs, which were shown to function in modulation of neural development and differentiation, with possible effect in the intellectual disability phenotype [ 76 ].…”
Section: Lncrnas and Pathways Involved In Non-syndromic Intellectumentioning
confidence: 99%