Autosomal Recessive 2 Spinocerebellar Ataxia

Also Known As:

Spinocerebellar Ataxia, Autosomal Recessive 2; CPD III; CPD3; Cerebellar Granular Cell Hypoplasia and Mental Retardation, Congenital; Cerebellar Hypoplasia, Nonprogressive Norman Type; Cerebelloparenchymal Disorder III; SCAR2; Spinocerebellar Ataxia Autosomal Recessive 2

Networked: 29 relevant articles (0 outcomes, 4 trials/studies)

Disease Context: Research Results

Related Diseases

1.	autosomal recessive 1 Spinocerebellar ataxia
2.	Cicatrix (Scar)
3.	Wounds and Injuries (Trauma)

Experts

1.	Mallery, Eileen L: 2 articles (01/2021 - 02/2005)
2.	Szymanski, Daniel B: 2 articles (01/2021 - 02/2005)
3.	Machesky, Laura M: 2 articles (02/2007 - 02/2003)
4.	Bouchard, Corinne: 1 article (05/2022)
5.	Bélanger, Sarah: 1 article (05/2022)
6.	Cardinal, Sébastien: 1 article (05/2022)
7.	Grenier, Alexe: 1 article (05/2022)
8.	Pouliot, Roxane: 1 article (05/2022)
9.	Voyer, Normand: 1 article (05/2022)
10.	Dang, Run: 1 article (01/2022)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Autosomal Recessive 2 Spinocerebellar Ataxia:

1.	Actin-Related Protein 2-3 Complex (Arp2 3 Complex)IBA 02/01/2005 - "DISTORTED3/SCAR2 is a putative arabidopsis WAVE complex subunit that activates the Arp2/3 complex and is required for epidermal morphogenesis." 02/01/2003 - "Furthermore, in NIH 3T3 cells, Scar2 and the Arp2/3 complex appear to be involved in the establishment and maintenance of Golgi polarity during directed migration." 02/01/2007 - "Here, we study the involvement of Scar1, Scar2, N-WASP, and Arp2/3 complex in dorsal ruffle formation in mouse embryonic fibroblasts (MEFs). " 02/01/2007 - "We hypothesize that N-WASP and Arp2/3 complex are part of a multiprotein assembly important for the generation of dorsal ruffles and that Scar1 and Scar2 are dispensable for this process." 02/01/2003 - "Involvement of the Arp2/3 complex and Scar2 in Golgi polarity in scratch wound models."
2.	culekinin depolarizing peptide IIIBA 11/01/1987 - "An excess of the substrate, however, is degraded by a ferric LPO----cpd I----cpd II----cpd III----ferrous LPO----ferric LPO cycle. " 01/01/2007 - "In this study, three 1H-benzimidazole derivatives with different electronic characteristics at position 5-, namely 5-chloro-4-(1H-benzimidazole-2-yl)phenol (Cpd I), 5-methyl-4-(1H-benzimidazole-2-yl)phenol (Cpd II) and 4-(1H-benzimidazole-2-yl)phenol (Cpd III), were synthesized and evaluated for their effects on mammalian type I DNA topoisomerase activity using quantitative in vitro plasmid supercoil relaxation assays. "
3.	Type I DNA Topoisomerases (Topoisomerase I)IBA 01/01/2007 - "In this study, three 1H-benzimidazole derivatives with different electronic characteristics at position 5-, namely 5-chloro-4-(1H-benzimidazole-2-yl)phenol (Cpd I), 5-methyl-4-(1H-benzimidazole-2-yl)phenol (Cpd II) and 4-(1H-benzimidazole-2-yl)phenol (Cpd III), were synthesized and evaluated for their effects on mammalian type I DNA topoisomerase activity using quantitative in vitro plasmid supercoil relaxation assays. "
4.	tesmilifene (DPPE)IBA 01/01/2018 - "In previous studies involving the following heteroleptic Ru complexes, [Ru(pic)(dppb)(bipy)]PF6 (SCAR1), [Ru(pic)(dppb)(Me-bipy)]PF6 (SCAR2), [Ru(pic)(dppb)(phen)]PF6 (SCAR4), cis-[Ru(pic)(dppe)2]PF6 (SCAR5), and [Ru(pic)(dppe)(phen)]PF6 (SCAR7), we observed excellent anti-TB activity, moderate cell-toxicity, and a lack of oral bioavailability in an in vivo model of these complexes. "
5.	Proteins (Proteins, Gene)FDA Link 01/01/2019 - "We report the identification of a novel compound (CPD3.1) with the ability to disrupt YAP-TEAD protein-protein interaction and inhibit TEAD activity, cell proliferation, and cell migration. " 10/01/2015 - "Although the SCARN protein is related to Arabidopsis thaliana SCAR2 and SCAR4, it belongs to a distinct legume-sub clade. " 07/30/2001 - "Co-expression experiments in aleurones suggested that the OsDof3 protein should play a regulatory role in the expression of the CPD3 gene under the control of GA." 01/01/2021 - "Here, we show that SPIRRIG (SPI), a beige and Chediak Higashi domain-containing protein involved in membrane trafficking, and BRK1 and SCAR2, subunits of the WAVE/SCAR (W/SC) actin nucleating promoting complex, display polarized localizations in Arabidopsis thaliana root hairs during distinct developmental stages. " 11/02/2020 - "Here, we demonstrate how API, a SCAR2 protein component of the SCAR/WAVE complex, controls the root cell wall architecture important for pathogenic oomycete and symbiotic bacterial interactions in legumes. "
6.	Hydrogen Peroxide (Hydroperoxide)FDA LinkGeneric 07/01/2002 - "When hydrogen peroxide is used in a >100 fold excess, the heme group is irreversibly destroyed, and even the characteristic band of cpd III is not found. " 08/01/1986 - "In the presence of iodide (I-, 10 mM) and hydrogen peroxide in a large excess (H2O2, 0.1-10 mM) catalytic amounts of lactoperoxidase (2 nM) are very rapidly irreversibly inactivated without forming compound III (cpd III). "
7.	EnzymesIBA 09/01/2018 - "In contrast to previous observation, Cpd3 did not inhibit Fe-SOD within trypanosomes and Cpd2 inhibited the enzyme only by 34%. " 09/01/2018 - "Cpd3 was also shown to be a potent inhibitor of trypanosomal iron superoxide dismutase (Fe-SOD) and its trypanocidal activity linked to the inhibition of this enzyme. " 07/30/2001 - "Type III carboxypeptidase (CPD3) is one of the hydrolytic enzymes whose expression is up-regulated by gibberellins (GA) in the aleurones of germinated cereal grains. "
8.	BrassinosteroidsIBA 02/01/2023 - "Both phyE and darkness were sufficient to repress the formation of somatic embryos and reduced the expression of CONSTITUTIVE PHOTOMORPHIC DWARF 3 (CPD3), a rate limiting step in brassinosteroid (BR) biosynthesis, as well as AGAMOUS LIKE 15 (AGL15), a key inducer of many SE genes. "
9.	Phospholipases A2 (Phospholipase A2)IBA 01/01/2022 - "Scratch activity was measured after oral administration of cpd3, in mice overexpressing phospholipase A2 ( sPLA2tg ), in wild type mice (WT) and in TRPA1-deficient mice (Trpa1 KO). "
10.	Amyloid Precursor Protein Secretases (beta-Secretase)IBA 01/01/2021 - "Here, the oto-regenerative potential of different γ-secretase inhibitors (GSIs) was evaluated in complementary assay models, including cell lines, organotypic cultures of the organ of Corti and cochlear organoids to characterize two novel GSIs (CPD3 and CPD8). "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 11/28/2023 - "Importantly, our study shows that the specific NR5A2 inhibitor, Cpd3, sensitizes a significant fraction of PDAC models derived from 18 patients to standard chemotherapy. "
2.	Artificial Skin 05/26/2022 - "Histological analyses of psoriatic skin substitutes treated with CPD1, CPD2 and CPD3 exhibited significantly reduced epidermal thickness compared with untreated psoriatic substitutes, which agreed with a decrease in keratinocyte proliferation as shown by Ki67 immunofluorescence staining. "
3.	Aftercare (After-Treatment) 01/01/2022 - "As expected, nociceptive behavior (induced by formalin, carrageenan or PGE2) was reduced after treatment with cpd3. "
4.	Oral Administration 01/01/2022 - "Scratch activity was measured after oral administration of cpd3, in mice overexpressing phospholipase A2 ( sPLA2tg ), in wild type mice (WT) and in TRPA1-deficient mice (Trpa1 KO). "