Dextran, Rhodamine B_RITC labeled, 20000 Da
- Known as:
- Dextran, Rhodamine B_RITC labeled, 20000 Da
- Catalog number:
- DX20-RB-1
- Product Quantity:
- 25 mg
- Category:
- -
- Supplier:
- Nanocs
- Gene target:
- Dextran Rhodamine B_RITC labeled 20000
Related genes to: Dextran, Rhodamine B_RITC labeled, 20000 Da
- Gene:
- KPTN NIH gene
- Name:
- kaptin, actin binding protein
- Previous symbol:
- -
- Synonyms:
- 20000
- Chromosome:
- 19q13.32
- Locus Type:
- gene with protein product
- Date approved:
- 1999-08-27
- Date modifiied:
- 2016-06-13
Related products to: Dextran, Rhodamine B_RITC labeled, 20000 Da
(Arg)9 biotin labeled(Arg)9, FAM - labeled(Arg)9, FAM - labeled(Arg)9, FAM - labeled(Arg)9, FAM - labeled(Arg)9, FAM - labeled(Arg)9, FAM _ labeled(Arg)9, FAM _ labeled(Arg)9, TAMRA - labeled(Z-AA)2Rh110[Rhodamine 110, bis-(CBZ-L-alanyl-L-alanine amide)](Z-Ala-Ala-Ala-Ala)2Rh110[Rhodamine 110, bis-(CBZ-L-alanyl-L-alanyl-L-alanyl-L- alanine amide)](Z-AR)2Rh110.HCl[Rhodamine 110, bis-(CBZ-L-alanyl-L-arginine amide)](Z-Arg)2Rh110.2HCl[Rhodamine 110, bis-(CBZ-L-arginine amide)]13C labeled13C labeled Related articles to: Dextran, Rhodamine B_RITC labeled, 20000 Da
- Pathogenic variants in Kaptin (KPTN) cause KPTN-related disorder (KRD). KPTN modulates mTOR signaling activation within the KICSTOR complex in response to cellular amino acid levels. We define the clinical spectrum and investigate the developmental pathogenesis of KRD. - Source: PubMed
Publication date: 2026/02/16
Rawlins Lettie EIffland Philip HPage JohnFlessner Rebecca ZElziny Soad MSbornova IrinaBabus Janice KBruckmeier Sophie RParikh RiaVerhoeven MerelFasham JamesLeslie Joseph SCaswell RichardUbeyratna NishankaWenger OliviaScott Ethan MSchreiber JohnSyrbe SteffenKlabunde-Cherwon AnnickOwens MartinaCrosby Andrew HBaple Emma LCrino Peter B - Mechanistic target of rapamycin complex 1 (mTORC1) is a master controller of cell growth, and its dysregulation is associated with cancer. KICSTOR, a complex comprising KPTN, ITFG2, C12orf66, and SZT2, functions as a critical negative regulator of amino acid-induced mTORC1 activation. However, the regulatory mechanisms governing KICSTOR remain largely unclear. In this study, we identify F-box only protein 2 (FBXO2) as a key modulator of amino acid-dependent mTORC1 signaling. Mechanistically, FBXO2 colocalizes and directly interacts with KPTN via its F-box-associated domain, promoting K48- and K63-linked polyubiquitination of KPTN at lysine residues 49, 67, 262, and 265. FBXO2-mediated KPTN ubiquitination disrupted its interaction with ITFG2 and SZT2, while enhancing its interaction with C12orf66, thereby impairing the ability of KICSTOR to recruit the GATOR1 complex - comprising DEPDC5, NPRL2, and NPRL3 - to the lysosomal surface. Notably, FBXO2 protein levels were substantially upregulated in patients with liver cancer, and FBXO2-mediated KPTN ubiquitination facilitated the progression of hepatocellular carcinoma (HCC). These results reveal a key regulatory mechanism of mTORC1 signaling and highlight FBXO2 and KPTN ubiquitination as therapeutic targets for HCC treatment. - Source: PubMed
Publication date: 2025/12/16
Gao JianfangQing JinaLi XianglongLuo YuxuanHuang LingwenLi HongxiaZhang HuanZhang JiaoXiao PeiLi JinsongLi TingtingHe Shanping - Kaptin-actin binding protein (KPTN)-related disorder is a rare autosomal recessive neurogenetic syndrome resulting from mutations in the KPTN gene, encoding the actin-binding protein kaptin. Symptoms experienced by individuals with KPTN-related disorder vary, but common features include intellectual disability, developmental delay, psychiatric manifestations, neonatal hypotonia, macrocephaly, and seizures. This case report describes two siblings from the United States affected by KPTN-related disorder, patient B (a 32-year-old woman) and patient M (a 30-year-old man). Both patients B and M have intellectual impairment, have had developmental delay/regression, and exhibit a range of neuropsychiatric symptoms. Additionally, both patients B and M experience seizures, with patient B primarily having absence seizures, and patient M having frequent myoclonic seizures. Interestingly, both patients discussed here maternally inherited a novel KPTN mutation, not previously identified in other individuals with KPTN-related disorder, to our knowledge. Of particular interest in this case is that several members of the maternal family were observed to have myoclonic seizures, raising the intriguing possibility that this newly identified KPTN mutation may lead to mild symptoms even in heterozygous carriers of this mutation. Furthermore, we discuss the recent diagnosis of both patients with sleep apnea, a feature not previously identified in other patients with KPTN-related disorder. - Source: PubMed
Publication date: 2025/12/04
Biswas IndraniFleet AlexRastanawie HamzaYang ShilinDeif AliBernad Peter G - Malformations of cortical development (MCD) caused by variants in mTOR pathway genes (MPGs) are a leading cause of drug-resistant epilepsy. Characteristic histopathological features of MPG-associated MCD include cytomegaly and cortical dyslamination often with neurons in abnormally close apposition (aggregates). We hypothesized that cellular aggregation is an mTOR-dependent phenotype. , or were knocked out (KO) using CRISPR/Cas9 in N2a cells . Levels of phosphorylated ribosomal S6 protein (PS6; Ser240/244), a marker for mTOR activation, were defined via Western blotting . Timelapse live-cell imaging was used to observe aggregate formation, with or without mTORC1 inhibition (rapamycin). EdU-base cell proliferation assay and cell death assays were performed to determine whether aggregation was the result of changes in cell cycle or increased cell death. Liquid chromatography-mass spectrometry (LC-MS/MS) was used to define changes in the cell aggregate proteome. Human MCD brain tissue specimens were stained with PS6 to assay mTOR signaling in neuronal clusters. All knockout lines formed multi-cell aggregates compared to control lines within 24-48 hours of plating . Aggregation was abolished with mTOR inhibitor treatment, establishing the mTOR-dependency of aggregate formation. Aggregation was not driven by cell proliferation, apoptosis/necrosis, or the presence of extracellular DNA in culture media. LC-MS/MS analysis revealed altered expression of protein across KO lines including adhesion molecules (e.g., contactin-3), cytoskeletal proteins (e.g., stathmin-2), and protein processing/transport (e.g., Uevld). Our findings establish aberrant cellular aggregation as an mTOR-dependent phenotype across multiple MPG associated with MCD. Changes in expression of adhesion molecules may contribute to abnormal cell aggregation and cortical lamination in MCD and results in abnormal network formation that leads to seizures. - Source: PubMed
Publication date: 2025/11/04
Roark Kelley MCrino Peter BIffland Philip H - Precise regulation of the actin cytoskeleton is fundamental to cellular morphology, motility, and intracellular transport. While key classes of actin-binding proteins, including nucleators, capping proteins, and bundlers, have been well characterized, additional modulators likely contribute to the spatial and temporal control of actin dynamics. Here, we identify Kaptin (KPTN), a protein localized to actin-rich structures at the cell periphery, as a novel regulator of actin filament dynamics. Using biochemical reconstitution and single-molecule TIRF microscopy, we demonstrate that KPTN binds to actin filament barbed ends and suppresses filament elongation. This activity leads to filament stabilization and bundling, suggesting a dual role in filament architecture maintenance. Structural prediction via AlphaFold classifies KPTN within the WD-repeat-containing protein family and highlights a conserved, positively charged residue within its predicted N-terminal β-propeller domain as essential for actin interaction. These findings uncover a novel mechanism by which KPTN regulates actin dynamics and establish it as both a barbed-end and side-binding protein within the actin cytoskeletal network. - Source: PubMed
Publication date: 2025/11/20
Dutta PriyankaMaiti IpshitaMondal Krishna ChandraGhose AurnabChauhan RadhaMaiti Sankar