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
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Related articles to: Coomassie brilliant blue R_250 2 xSolution
- 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 - Polycystic liver and kidney disease encompasses a spectrum of inherited disorders ,primarily autosomal dominant polycystic kidney disease (ADPKD) and autosomal dominant polycystic liver disease (ADPLD). Although many patients remain asymptomatic for decades, a subset develops debilitating complications, including massive hepatomegaly, recurrent cyst infections, malnutrition, and progressive renal dysfunction. Diagnosis relies on imaging criteria and genetic testing. ADPKD is most commonly associated with PKD1 and PKD2 mutations, whereas ADPLD is associated with PRKCSH and SEC63 mutations. Magnetic resonance imaging provides optimal assessment of cyst distribution, liver volume, and complications. Disease severity classifications, such as Schnelldorfer classification, guide therapeutic decision-making. Liver transplantation is the only definitive treatment for severe symptomatic PLD. Primary indications include recurrent cyst infections refractory to medical therapy, massive hepatomegaly with significant impairment of quality of life, malnutrition, and portal hypertension. Patient-reported instruments, including PLD-Q and POLCA, questionnaires, provide objective measures of symptom burden and aid in transplant candidacy assessment. In patients with concurrent renal insufficiency (estimated glomerular filtration rate <30 mL/min/1.73 m2), combined liver–kidney transplantation may improve outcomes and confer potential immunoprotective benefits for the renal graft. Despite favorable posttransplant outcomes—including 5-year survival rates exceeding 80%—variation in listing criteria persists, highlighting the need for standardized, evidence-based guidelines. Multidisciplinary evaluation and individualized management remain essential to optimize clinical outcomes in this complex patient population. - Source: PubMed
Publication date: 2026/05/20
Perez-Prado SandraSuddle Abid - Genetic disease is often unrecognised. This study examined how often renal clinic patients, with a non-genetic diagnosis and without a family history of genetic disease recorded at the last three visits, had an underlying genetic form of kidney disease or diabetes. Sixty-four consecutive unrelated patients from a general nephrology clinic (44 male, 20 female) with a median age of 66 years (range 26 - 87) underwent chart review and Whole Exome Sequencing (WES). WES results were filtered for rare disease-causing single nucleotide and copy number variants in genetic kidney disease (n = 495) and monogenic diabetes (n = 8) genes. The patients' diagnoses included diabetes (n = 36, 56%), diabetic kidney disease (n = 28, 44%), autoimmune glomerulonephritis (n = 20,31%) or others (n = 12, 19%). Six (9%) volunteered a family history of genetic disease at recruitment. Twenty had at least one kidney cyst on imaging (20/59, 34%). Forty-four (69%) had an eGFR between 20 and 60 ml/min/1.73m, and 14 (22%) an eGFR < 20 ml/min/1.73m or had undergone renal replacement therapy. Seven Pathogenic or Likely pathogenic variants (according to ClinVar) were found in 7 people (11%), in COL4A3, CFH, and CHD7, and 3 cystic kidney genes (PKHD1 (n = 2), SEC63, LRP5). Three further individuals with diabetic kidney disease (3/36, 8%) had a possibly pathogenic variant (Likely pathogenic in one, and VUS in 2, in ClinVar) in a monogenic diabetes gene (HNF1A, ABCC8, NEUROD1). Pathogenic variants in a gene associated with kidney disease were identified in only three of the 6 patients with a positive family history. In conclusion, genetic variants associated with monogenic kidney disease occurred in possibly 11% (7/64) of renal clinic patients with a non-genetic diagnosis, and variants associated with monogenic diabetes in a further 9% (3/36) with diabetic kidney disease. - Source: PubMed
Publication date: 2026/05/15
Huang MaryPianta TimothyBarit DavidMahmood KhalidColville DebMack HeatherSavige Judy