Results 61 to 70 of about 2,984,931 (176)
ABSTRACT Modern biopharmaceutical manufacturing requires purification platforms capable of processing structurally and functionally diverse products while addressing the challenge of removing persistent and high‐risk host cell proteins (HCPs).
Wenning Chu +23 more
wiley +1 more source
CT features of Wolman disease (lysosomal acid lipase enzyme deficiency) – A case report
Wolman disease is a lethal rare autosomal recessive disorder defined by the deficiency of acid lipase enzyme. The disease is a lysosomal storage disease. Multiple organs such as adrenal glands, liver, spleen, bone marrow, small bowel loops, and abdominal
Naqibullah Foladi, MD +1 more
doaj +1 more source
ABSTRACT Persistence of HCPs remains a major purification challenge in biopharmaceutical manufacturing with direct consequences for product quality, patient safety, and regulatory compliance. Over the last two decades, deeper insights into the mechanisms driving HCP persistence have clarified why certain proteins evade removal during purification ...
Younghoon Oh +10 more
wiley +1 more source
This review highlights critical “translational gaps” in understanding micro‐ and nanoplastics impacts on gastrointestinal diseases, bridging exposure, detecting, and mechanistic toxicology. Abstract Micro‐ and nanoplastics (MNPs) have become pervasive environmental contaminants with increasing evidence linking them to adverse health outcomes.
Zhenli Diao +3 more
wiley +1 more source
Lysosomal acid lipase deficiency (LAL‐D) is an autosomal recessive disease characterized by hypoalphalipoproteinemia, mixed hyperlipemia, and fatty liver (FL) due to mutations in LIPAse A, lysosomal acid type (LIPA) gene.
Andrea Pasta +11 more
doaj +1 more source
Status and future of recombinant adeno‐associated virus vector manufacturing
Abstract Sixty years of adeno‐associated virus (AAV) research illustrates a trajectory marked by basic science exploration, iterative innovation, persistent challenges, a number of clinical setbacks, as well as commercial therapeutic triumphs. This continual evolution has led to recombinant AAV (rAAV) becoming a cornerstone of modern gene therapy ...
Frank Agbogbo, David Dismuke
wiley +1 more source
Hepatic Cholesteryl Ester Accumulation in Lysosomal Acid Lipase Deficiency: Non-Invasive Identification and Treatment Monitoring by Magnetic Resonance [PDF]
Background & Aims: Lysosomal Acid Lipase (LAL) deficiency is a rare metabolic storage disease, caused by a marked reduction in activity of LAL, which leads to accumulation of cholesteryl esters (CE) and triglycerides (TG) in lysosomes in many tissues.
Thoma C +11 more
core +5 more sources
Objectives: To determine the prevalence of Lysosomal Acid Lipase (LAL) deficiency in patients with severe dyslipidemia and premature coronary heart ...
Marmelo, B. +10 more
core +1 more source
Lysosomes play a key role in the accumulation, catabolism, and transport of endogenous and exogenous metabolites and proteins and are involved in drug metabolism and prodrug activation. However, the protein abundance and interindividual variability of lysosomal drug‐metabolizing enzymes and transporters (DMETs) remain underexplored.
Darshak Gadara +20 more
wiley +1 more source
Lysosomal acid lipase deficiency in children: literature review and clinical observations
The enzyme lysosomal acid lipase, encoded by the LIPA gene, plays a key role in lipid metabolism in lysosomes. Mutations in the LIPA gene, of which about 120 have been registered, lead to a ...
G. V. Volynets, A. S. Potapov
core +1 more source

