Gian Carlo Demontis
Overview
Explore the profile of Gian Carlo Demontis including associated specialties, affiliations and a list of published articles.
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Articles
28
Citations
390
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Recent Articles
1.
Andreazzoli M, Longoni B, Angeloni D, Demontis G
Cells
. 2024 May;
13(10.
PMID: 38786093
Vision starts in retinal photoreceptors when specialized proteins (opsins) sense photons via their covalently bonded vitamin A derivative 11cis retinaldehyde (11cis-RAL). The reaction of non-enzymatic aldehydes with amino groups lacks...
2.
Barravecchia I, De Cesari C, Guadagni V, Signore G, Bertolini E, Giannelli S, et al.
Sci Rep
. 2023 Apr;
13(1):6025.
PMID: 37055439
In proliferating multipotent retinal progenitors, transcription factors dynamics set the fate of postmitotic daughter cells, but postmitotic cell fate plasticity driven by extrinsic factors remains controversial. Transcriptome analysis reveals the...
3.
Longoni B, Demontis G
Antioxidants (Basel)
. 2023 Mar;
12(3).
PMID: 36978865
The retina is an oxidative stress-prone tissue due to high content of polyunsaturated lipids, exposure to visible light stimuli in the 400-480 nm range, and high oxygen availability provided by...
4.
Poli G, Demontis G, Sodi A, Saba A, Rizzo S, Macchia M, et al.
J Enzyme Inhib Med Chem
. 2023 Jan;
38(1):2162047.
PMID: 36629452
RPE65 is a fundamental enzyme of the retinoid visual cycle, and many missense mutations affecting its expression or function are associated with a wide range of diseases. Many RPE65 missense...
5.
Vellani V, Mauro G, Demontis G
Int J Mol Sci
. 2022 Nov;
23(21).
PMID: 36361866
Unidentified pathogenetic mechanisms and genetic and clinical heterogeneity represent critical factors hindering the development of treatments for inherited retinal dystrophies. Frameshift mutations in , which codes for an accessory subunit...
6.
Poli G, Barravecchia I, Demontis G, Sodi A, Saba A, Rizzo S, et al.
J Enzyme Inhib Med Chem
. 2022 Jun;
37(1):1765-1772.
PMID: 35726567
The human retinal pigment epithelium-specific 65-kDa protein (RPE65) plays a crucial role within the retinoid visual cycle and several mutations affecting either its expression level or its enzymatic function are...
7.
Andreazzoli M, Barravecchia I, De Cesari C, Angeloni D, Demontis G
Cells
. 2021 Sep;
10(9).
PMID: 34572137
Inherited retinal degenerations (IRD) affecting either photoreceptors or pigment epithelial cells cause progressive visual loss and severe disability, up to complete blindness. Retinal organoids (ROs) technologies opened up the development...
8.
Bononi G, Tonarini G, Poli G, Barravecchia I, Caligiuri I, Macchia M, et al.
Eur J Med Chem
. 2021 Jul;
223:113679.
PMID: 34218085
Monoacylglycerol lipase (MAGL) is an enzyme belonging to the endocannabinoid system that mainly metabolizes the endocannabinoid 2-arachidonoylglycerol (2-AG). Numerous studies have shown the involvement of this enzyme in various pathological...
9.
Barravecchia I, Demontis G
Prog Biophys Mol Biol
. 2021 Jul;
166:133-146.
PMID: 34197835
Most primary sensory neurons (PSNs) generate a slowly-activating inward current in response to membrane hyperpolarization (I) and express HCN1 along with additional isoforms coding for hyperpolarization-activated channels (HCN). Changes in...
10.
Demontis G, Pezzini F, Margari E, Bianchi M, Longoni B, Doccini S, et al.
Front Cell Neurosci
. 2021 Jan;
14:569598.
PMID: 33390903
CLN1 disease (OMIM #256730) is an inherited neurological disorder of early childhood with epileptic seizures and premature death. It is associated with mutations in coding for Palmitoyl-Protein Thioesterase 1 (PPT1),...