Disorders of sterol biosynthesis

Furtado, Larissa V.; Kelley, Richard I.; Opitz, John M.

doi:10.3233/trd-160004

Cited by 2 publications

(2 citation statements)

References 209 publications

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“…SQLE (squalene epoxidase) is rate limiting and the first oxygenation enzyme in cholesterol synthesis [61]. Lanosterol, especially cholesterol, is the upstream precursor of sterol biosynthesis in fungal steroids and animals [62]. Herein, hypo-salinity up-regulated sqle, ebp, and lss in the gills, indicating the stimulation of cholesterol synthesis in the gills.…”

Section: Degs Involved In Steroid Biosynthesis and Lipid Metabolismmentioning

confidence: 99%

RNA Sequencing Analysis Reveals Divergent Adaptive Response to Hypo- and Hyper-Salinity in Greater Amberjack (Seriola dumerili) Juveniles

Peng

Shi

Liu

et al. 2022

Animals

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Salinity significantly affects physiological and metabolic activities, breeding, development, survival, and growth of marine fish. The greater amberjack (Seriola dumerili) is a fast-growing species that has immensely contributed to global aquaculture diversification. However, the tolerance, adaptation, and molecular responses of greater amberjack to salinity are unclear. This study reared greater amberjack juveniles under different salinity stresses (40, 30, 20, and 10 ppt) for 30 days to assess their tolerance, adaptation, and molecular responses to salinity. RNA sequencing analysis of gill tissue was used to identify genes and biological processes involved in greater amberjack response to salinity stress at 40, 30, and 20 ppt. Eighteen differentially expressed genes (DEGs) (nine upregulated and nine downregulated) were identified in the 40 vs. 30 ppt group. Moreover, 417 DEGs (205 up-regulated and 212 down-regulated) were identified in the 20 vs. 30 ppt group. qPCR and transcriptomic analysis indicated that salinity stress affected the expression of genes involved in steroid biosynthesis (ebp, sqle, lss, dhcr7, dhcr24, and cyp51a1), lipid metabolism (msmo1, nsdhl, ogdh, and edar), ion transporters (slc25a48, slc37a4, slc44a4, and apq4), and immune response (wnt4 and tlr5). Furthermore, KEGG pathway enrichment analysis showed that the DEGs were enriched in steroid biosynthesis, lipids metabolism, cytokine–cytokine receptor interaction, tryptophan metabolism, and insulin signaling pathway. Therefore, this study provides insights into the molecular mechanisms of marine fish adaptation to salinity.

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Section: Degs Involved In Steroid Biosynthesis and Lipid Metabolismmentioning

confidence: 99%

RNA Sequencing Analysis Reveals Divergent Adaptive Response to Hypo- and Hyper-Salinity in Greater Amberjack (Seriola dumerili) Juveniles

Peng

Shi

Liu

et al. 2022

Animals

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“…Indeed, several genetic disorders of the cholesterol biosynthetic pathway are associated with demyelination with or without craniofacial malformations and accumulation of sterol intermediate precursors [ 117 , 138 ]. OLs produce the bulk of cholesterol incorporated into myelin and, in the case of a deficiency in cholesterol biosynthesis, an efficient horizontal transfer of cholesterol between different brain cell types has been observed.…”

Section: Shh Signaling Cholesterol Biosynthesis and Myelination: mentioning

confidence: 99%

Targeting Smoothened as a New Frontier in the Functional Recovery of Central Nervous System Demyelinating Pathologies

Giovane

Ragnini-Wilson

2018

IJMS

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Myelin sheaths on vertebrate axons provide protection, vital support and increase the speed of neuronal signals. Myelin degeneration can be caused by viral, autoimmune or genetic diseases. Remyelination is a natural process that restores the myelin sheath and, consequently, neuronal function after a demyelination event, preventing neurodegeneration and thereby neuron functional loss. Pharmacological approaches to remyelination represent a promising new frontier in the therapy of human demyelination pathologies and might provide novel tools to improve adaptive myelination in aged individuals. Recent phenotypical screens have identified agonists of the atypical G protein-coupled receptor Smoothened and inhibitors of the glioma-associated oncogene 1 as being amongst the most potent stimulators of oligodendrocyte precursor cell (OPC) differentiation in vitro and remyelination in the central nervous system (CNS) of mice. Here, we discuss the current state-of-the-art of studies on the role of Sonic Hedgehog reactivation during remyelination, referring readers to other reviews for the role of Hedgehog signaling in cancer and stem cell maintenance.

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Disorders of sterol biosynthesis

Cited by 2 publications

References 209 publications

RNA Sequencing Analysis Reveals Divergent Adaptive Response to Hypo- and Hyper-Salinity in Greater Amberjack (Seriola dumerili) Juveniles

RNA Sequencing Analysis Reveals Divergent Adaptive Response to Hypo- and Hyper-Salinity in Greater Amberjack (Seriola dumerili) Juveniles

Targeting Smoothened as a New Frontier in the Functional Recovery of Central Nervous System Demyelinating Pathologies

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