Coomassie brilliant blue R_250 2 xSolution
- Known as:
- Coomassie brilliant blue R_250 2 xSolution
- Catalog number:
- C462151
- Product Quantity:
- 250ML
- Category:
- -
- Supplier:
- BioBasic
- Gene target:
- Coomassie brilliant blue R_250 2 xSolution
Related products to: Coomassie brilliant blue R_250 2 xSolution
Related articles to: Coomassie brilliant blue R_250 2 xSolution
- Autophagic degradation of parts of the nucleus (nucleophagy) and endoplasmic reticulum (ER-phagy) utilizes the selective autophagy receptors, Atg39 and Atg40, that link their target structures to the autophagy machinery. Here, we show that a complex of three proteins, Apq12, Brr6, and Brl1, is needed for both Atg39-dependent nucleophagy and Atg40-dependent ER-phagy. Apq12, Brr6, and Brl1 each have two transmembrane domains flanking a luminal region that contains an amphipathic helix. The Apq12/Brr6/Brl1 complex has been implicated in membrane remodeling during nuclear pore formation. While autophagosomal flux is unaffected by the loss of Apq12, Brr6, or Brl1, vacuolar delivery of outer and inner nuclear membrane proteins (Hmg1, Nvj1, and Src1), pan-ER proteins (Sec63 and Per33), and an ER tubule junction protein (Lnp1) is blocked. In cells, Atg39 and Atg40 receptors concentrate in puncta and associate with the autophagic machinery; however, their delivery to the vacuole is blocked. Atg39 puncta extend into the cytoplasm but fail to detach from the nuclear envelope (NE), suggesting a defect in membrane scission. Coimmunoprecipitation and crosslinking analysis indicate that Atg39 and Atg40 interact with the Apq12/Brr6/Brl1 complex. We propose that the membrane remodeling activity of the Apq12/Brr6/Brl1 complex is needed to release fragments of the NE and ER so they can be sequestered within autophagosomes for degradation. - Source: PubMed
Publication date: 2026/09/30
Liu DongmeiChen ShuliangYuan HuaFerro-Novick SusanNovick Peter - Autosomal dominant polycystic liver disease (ADPLD) commonly results from pathogenic variants in one of 7 genes (GANAB, ALG8, ALG9, PRKCSH, SEC63 and possibly LRP5 and SEC61B). These genes are also included in kidney panels because pathogenic variants cause kidney cysts but rarely kidney failure. This study determined the population frequency of predicted pathogenic variants in the ADPLD genes in the general population. Variants for each gene were downloaded from gnomADv.2.1.1 and annotated with ANNOVAR. The population frequencies were calculated from the number of people with 'predicted pathogenic' variants in gnomAD: loss-of-function structural and copy number; null; and rare, computationally-damaging missense changes that affected a conserved residue. Frequencies were also estimated from the number of gnomADv.4.1 variants assessed as Pathogenic or Likely pathogenic in ClinVar. Predicted pathogenic variants affected one in 91 people using our strategy and gnomAD v.2.1.1, and one in 130 with ClinVar assessments of gnomAD v.4.1 variants. LRP5 and ALG8 which are associated with a milder clinical phenotype, were the commonest affected genes with both strategies. Predicted pathogenic variants in ADPLD appear more frequent in admixed American (one in 91), Finnish (one in 110) and African/African American (one in 43) people (p all < 0.0001 compared with Europeans (one in 187). Predicted pathogenic variants for ADPLD may be even more common with the finding of additional causative genes. Assessments will become more accurate too. However not all ADPLD variants result in liver cysts, nor indeed cystic kidneys, because of incomplete penetrance and variable expressivity. - Source: PubMed
Publication date: 2026/09/28
Varughese SantoshHuang MarySavige Judy - The Sec61 complex plays a critical role in protein translocation into the endoplasmic reticulum (ER), enabling the passage of soluble proteins into the lumen and the lateral insertion of membrane proteins. The Sec complex, comprising Sec61, Sec63, Sec62, and other auxiliary proteins, is especially important for the translocation of precursor proteins with inefficient signal sequences (SSs) that require additional assistance to trigger the opening of the Sec61 channel. In this study, we investigated how the hydrophobicity of SSs affects the interaction with the Sec complex at the single-molecule level using optical tweezers. We analyzed three SSs representing low, moderate, and high hydrophobicity in the h-regions. Our results show that the highly hydrophobic SS from DPAP-B pαF forms a less stable bond with the Sec complex, evidenced by shorter interaction time and reduced energy barrier to the unbound state. In contrast, SSs with lower hydrophobicity establish more stable interactions, characterized by extended interaction times, and elevated energy barriers to reach the unbound state. These findings suggest that the Sec complex may selectively recognize and interact with SSs based on their hydrophobicity, offering insights into the mechanisms by which defects in SS translocation contribute to ER-associated diseases. - Source: PubMed
Publication date: 2026/08/01
Sánchez Wendy NRobeson LukaAlfaro-Valdés Hilda MTych KatarzynaMatute Ricardo ADriessen Arnold J MWilson Christian A M - Polycystic liver disease and other manifestations of ductal plate malformation (DPM) may be incidental or indeed develop significant complications. The genetic basis of 'isolated' DPM differs from that associated with polycystic kidney disease and remains incompletely understood. - Source: PubMed
Publication date: 2026/06/28
Bhuva MehaThompson EdwardWatson Christopher MSava FlorentinaGriffiths William J H - The present study aimed to assess the conservation status and characterize the phenotypic and genomic diversity of two endangered Italian poultry genetic resources to guide their conservation plans. Blood samples and morphometric data were collected on 50 Faraona Camosciata (FAC, Numida meleagris) and 50 Oca Padovana (OPD, Anser anser) adult animals. Genomes were profiled by low coverage whole genome sequencing. Observed and expected heterozygosity, genomic inbreeding, population structure, runs of homozygosity (ROH), ROH islands, and Gene Ontology enrichment were evaluated. Morphometric measures revealed significant sexual dimorphism within both breeds. FAC females exceeded males for several traits, and OPD males exceeded females for most features. Both breeds had very low heterozygosity and high inbreeding, consistent with their small effective sizes. The ROH length spectra indicated distinct histories: FAC was enriched in numerous short tracts under 2 Mb, compatible with older, diffuse autozygosity, and OPD carried a higher burden of long tracts between 4 and 16 Mb, consistent with more recent common ancestry. Recurrent ROH islands were detected in both breeds. In FAC, islands were enriched for hydrolase and catabolic functions, and organelle related components, including genes such as RAD54L, SEC63, and POMGNT1. In OPD, islands emphasized kinase regulation, ion channel and contractile complexes, and endocrine metabolism, featuring IGF2, INS, TH, and GJA1 genes. Results provide a baseline for genetic management that prioritizes avoidance of matings among relatives, rotation of animals across centers, balanced contributions, and monitoring through genetic methods, while underscoring the importance of improved genome annotation for these non-model species. - Source: PubMed
Cendron FilippoBortoletto EnricoPerini FrancescoBaruchello MaristellaRosani UmbertoLasagna EmilianoCassandro MartinoPenasa Mauro