The Biology and Clinical Implications of PCSK7.

Discovered in 1996, PCSK7 is the seventh of the nine-membered proprotein convertase subtilisin-kexin (PCSK) family. This article reviews the various aspects of the multifaceted biology of PCSK7 and what makes it an exciting new target for metabolic dysfunction-associated steatotic liver disease (MASLD) affecting ∼30% of the population globally, dyslipidemia, cardiovascular disease (CVD), and likely cancer/metastasis. We will systematically review and discuss all the available epidemiological data, structural, cell biology, and in vivo evidence that eventually led to the discovery of PCSK7 as a novel post-translational regulator of apolipoprotein B.

The Use of Automated Insulin Delivery around Physical Activity and Exercise in Type 1 Diabetes: A Position Statement of the European Association for the Study of Diabetes (EASD) and the International Society for Pediatric and Adolescent Diabetes (ISPAD).

Regular physical activity and exercise (PA) are cornerstones of diabetes care for individuals with type 1 diabetes. In recent years, the availability of automated insulin delivery (AID) systems has improved the ability of people with type 1 diabetes to achieve the recommended glucose target ranges. PA provides additional health benefits but can cause glucose fluctuations, which challenges current AID systems.

The use of automated insulin delivery around physical activity and exercise in type 1 diabetes: a position statement of the European Association for the Study of Diabetes (EASD) and the International Society for Pediatric and Adolescent Diabetes (ISPAD).

Regular physical activity and exercise (PA) are cornerstones of diabetes care for individuals with type 1 diabetes. In recent years, the availability of automated insulin delivery (AID) systems has improved the ability of people with type 1 diabetes to achieve the recommended glucose target ranges. PA provide additional health benefits but can cause glucose fluctuations, which challenges current AID systems.

Extreme LDL-C concentration is associated with increased cardiovascular disease in women with homozygous familial hypercholesterolemia.

Background: Homozygous familial hypercholesterolemia (HoFH) is a rare genetic disease of low-density lipoprotein cholesterol (LDL-C) metabolism. Despite the devastating effect of this disease on atherosclerotic cardiovascular health, the disease phenotype and severity are more heterogeneous than previously thought. The predictors of atherosclerotic cardiovascular disease (ASCVD) in HoFH patients have never been systematically studied.

PCSK9 in metabolism and diseases.

PCSK9 is a serine protease that regulates plasma levels of low-density lipoprotein (LDL) and cholesterol by mediating the endolysosomal degradation of LDL receptor (LDLR) in the liver. When PCSK9 functions unchecked, it leads to increased degradation of LDLR, resulting in elevated circulatory levels of LDL and cholesterol. This dysregulation contributes to lipid and cholesterol metabolism abnormalities, foam cell formation, and the development of various diseases, including cardiovascular disease (CVD), viral infections, cancer, and sepsis.

The impact of exercise timing on energy intake: A systematic review and meta-analysis of diurnal and meal timing effects.

This systematic review and meta-analysis examine the literature (up to August 2nd 2024) on the influence of exercise timing on energy intake in both children and adults. A comprehensive search was conducted using MEDLINE, EMBASE, Cochrane Library, SPORTDiscus, and Web of Science Core Collection, following PRISMA guidelines. The review was registered in Prospero (CRD42024553381) and evaluated using QUADAS-2.

Corrigendum: Medical Assistance in Dying in Quebec: A Continuum Between Teams' Accountability and Interdisciplinary Support Groups' Assumption of Responsibility.

[This corrects the article DOI: 10.3389/ijph.2024.

A human DCC variant causing mirror movement disorder reveals that the WAVE regulatory complex mediates axon guidance by netrin-1-DCC.

The axon guidance cue netrin-1 signals through its receptor DCC (deleted in colorectal cancer) to attract commissural axons to the midline. Variants in DCC are frequently associated with congenital mirror movements (CMMs). A CMM-associated variant in the cytoplasmic tail of DCC is located in a conserved motif predicted to bind to a regulator of actin dynamics called the WAVE (Wiskott-Aldrich syndrome protein-family verprolin homologous protein) regulatory complex (WRC).

A Patient-Centered Perspective on Changes in Personal Characteristics After Deep Brain Stimulation.

Importance: Deep brain stimulation (DBS) results in improvements in motor function and quality of life in patients with Parkinson disease (PD), which might impact a patient's perception of valued personal characteristics. Prior studies investigating whether DBS causes unwanted changes to oneself or one's personality have methodological limitations that should be addressed.

Objective: To determine whether DBS is associated with changes in characteristics that patients with PD identify as personally meaningful.

Medical Assistance in Dying in Quebec: A Continuum Between Teams' Accountability and Interdisciplinary Support Groups' Assumption of Responsibility.

Objectives: In the province of Quebec, Canada, interdisciplinary support groups (ISGs) are mandated to support those who are involved in the clinical, administrative, legal and ethical aspects of medical assistance in dying (MAiD). This article presents the results of a mixed-method, multi-phase study carried out in 2021 on ISGs with the aim to describe current ISG practices, critically analyze them and make recommendations on promising practices for provincial implementation.

Method: Semi-structured interviews (42) and focus groups (7) with coordinators of 24 ISGs were used to identify promising practices and confirm their utility with participants.

Plozasiran for Managing Persistent Chylomicronemia and Pancreatitis Risk.

Background: Persistent chylomicronemia is a genetic recessive disorder that is classically caused by familial chylomicronemia syndrome (FCS), but it also has multifactorial causes. The disorder is associated with the risk of recurrent acute pancreatitis. Plozasiran is a small interfering RNA that reduces hepatic production of apolipoprotein C-III and circulating triglycerides.

Overweight and Obesity in People Living With Type 1 Diabetes: A Cross-Sectional Analysis of the BETTER Registry.

Aims: The prevalence and associations of overweight and obesity in Canadian adult people living with type 1 diabetes (PWT1D) are poorly documented. In a cohort of PWT1D patients, this study assesses (i) overweight and obesity frequencies and associated PWT1D clinicodemographic characteristics, (ii) diabetes characteristics, and (iii) the use of noninsulin adjunctive agents.

Materials And Methods: Cross-sectional analysis of self-reported data from the BETTER registry: 1091 adult PWT1D (aged 44.

Cystic fibrosis-related diabetes develops from a combination of insulin secretion defects and insulin resistance.

Aim: The relative contributions of insulin secretory defects and possible additional contribution of insulin resistance for the development of cystic fibrosis (CF)-related diabetes (CFRD) are poorly understood. We aimed to (a) determine which indices of insulin resistance predict progression to CFRD, and (b) to model the relative contributions of insulin secretory function and insulin resistance to predict the risk of CFRD.

Materials And Methods: Three hundred and three individuals living with CF underwent a 2-h oral glucose tolerance test with blood sampling every 30 min at 12-24-month intervals until they developed CFRD or until the end of follow-up (up to 15 years).

First family with Perry syndrome from Mexico.

Perry syndrome (PS) is a rare autosomal dominant disease characterized by parkinsonism, central hypoventilation, weight loss and depression and is caused by pathogenic mutations in the dynactin subunit 1 () gene (encoding p150 protein). To date, only two cases have been reported in Latin America, specifically in Colombia and Argentina. The present study, to the best of our knowledge, reports the first recorded Mexican family with PS.