Inappropriate accumulation of the misfolded Z variant of alpha1-antitrypsin in the hepatocyte endoplasmic reticulum (ER) is a risk factor for the development of end-stage liver disease. However, the genetic and environmental factors that contribute to its etiology are poorly understood. ER mannosidase I (ERManI) is a quality control factor that plays a critical role in the sorting and targeting of misfolded glycoproteins for proteasome-mediated degradation. In this study, we tested whether genetic variations in the human ERManI gene influence the age at onset of end-stage liver disease in patients homozygous for the Z allele (ZZ). We sequenced all 13 exons in a group of unrelated Caucasian ZZ transplant recipients with different age at onset of the end-stage liver disease. Homozygosity for the minor A allele at 2484G/A (refSNP ID number rs4567) in the 3′-untranslated region was prevalent in the infant ZZ patients. Functional studies indicated that rs4567(A), but not rs4567(G), suppresses ERManI translation under ER stress conditions. Conclusion: These findings suggest that the identified single-nucleotide polymorphism can accelerate the onset of the end-stage liver disease associated with alpha1-antitrypsin deficiency and underscore the contribution of biosynthetic quality control as a modifier of genetic disease. (Hepatology 2009.)

In this study, a multi-starters fermentation system involved lactic acid bacteria and yeasts was applied to obtain a novel acidified goat milk (AGM). Significant differences were found in the volatile flavor profile among goat milk, goat yogurt, and AGM reflected by principal component analysis of electronic nose (E-nose) data. Gas chromatography–mass spectrometry (GC-MS) results indicated that the relative content of free octanoic acid decreased, and more aromas were formed in AGM, which were considered to mask the goaty smell and give AGM a pleasant flavor. Rheological analysis indicated that AGM had higher apparent viscosity and G’ and G’’ moduli than goat yogurt and goat milk. Therefore, the goat yogurt fermented by lactic acid bacteria and K. marxianus exhibits a new method to alleviate the goaty flavor in goat milk and provides a novel option for those who were allergic to milk protein and dislike goaty flavor in goat milk.

Soybean (Glycine max (L.) Merrill) is a key source of plant protein, with 7S and 11S globulins being the primary fractions. This study investigated the protein content, protein composition, and 11S/7S globulin ratios of 411 soybean samples, and then selected six varieties (S1, S2, S3, S4, S5, and S6) for the analysis of the protein structures and functional properties. The results revealed that varieties with low 11S/7S ratios (S1, S2, and S3) exhibited relatively high random coil contents (20.11–22.94%) and lower β-sheet contents (34.17–38.37%), suggesting the presence of more loosely structured proteins. S2 showed good solubility (73.21%) and water-holding capacity (WHC) (2.73 g/g), which can improve product quality and yield. In contrast, varieties with high 11S/7S ratios (S4, S5, and S6) demonstrated more compact protein structures, increased surface hydrophobicity, larger particle sizes, lower absolute zeta potential values, and greater oil-holding capacity (OHC) values (7.58–8.48 g/g). S4, in particular, demonstrated superior emulsification properties, with emulsion activity index (EAI) (4.71 m²/g) and emulsion stability index (ESI) (58.73 min), which are widely used in the food industry such as in cake, ice cream, and bread. This study provides valuable information for the selection of soybean varieties with optimal 11S/7S ratios for processing soybean products.