Efficacy of Fluorinated Nematicides for Management of Root-knot Nematodes in California Processing Tomatoes.

California is the primary US producer of processing tomatoes. After decades-long excellent protection against the common tropical spp. , , and (root-knot nematode: RKN) by -resistant tomato cultivars, resistance-breaking RKN populations are spreading throughout the San Joaquin Valley.

Cipaglucosidase alfa plus miglustat: linking mechanism of action to clinical outcomes in late-onset Pompe disease.

Enzyme replacement therapy (ERT) is the only approved disease-modifying treatment modality for Pompe disease, a rare, inherited metabolic disorder caused by a deficiency in the acid -glucosidase (GAA) enzyme that catabolizes lysosomal glycogen. First-generation recombinant human GAA (rhGAA) ERT (alglucosidase alfa) can slow the progressive muscle degeneration characteristic of the disease. Still, most patients experience diminished efficacy over time, possibly because of poor uptake into target tissues.

Brain glycogen build-up measured by magnetic resonance spectroscopy in classic infantile Pompe disease.

Classic infantile Pompe disease is caused by abnormal lysosomal glycogen accumulation in multiple tissues, including the brain due to a deficit in acid α-glucosidase. Although treatment with recombinant human acid α-glucosidase has dramatically improved survival, recombinant human acid α-glucosidase does not reach the brain, and surviving classic infantile Pompe patients develop progressive cognitive deficits and white matter lesions. We investigated the feasibility of measuring non-invasively glycogen build-up and other metabolic alterations in the brain of classic infantile Pompe patients.

The efficacy of psychological interventions for posttraumatic stress disorder symptoms in incarcerated forensic populations: A meta-analysis of randomized controlled trials.

Objective: Given the high prevalence of posttraumatic stress disorder (PTSD) symptoms in incarcerated forensic populations, this meta-analysis estimated the efficacy of interventions in this field and explored predictors of intervention outcomes.

Method: Twelve randomized controlled trials of psychological interventions for PTSD symptoms in incarcerated forensic populations were included. A combined Hedges's g effect size was synthesized, quality assessments and risk of bias analyses were performed, and publication bias was assessed.

Long term survival in patients with classic infantile Pompe disease reveals a spectrum with progressive brain abnormalities and changes in cognitive functioning.

The aim of this longitudinal cohort study, is to provide more insight into the pattern of brain abnormalities, and possible consequences for cognitive functioning, in patients with classic infantile Pompe disease. We included 19 classic infantile Pompe patients (median age last assessment 8.9 years, range 1.

Host status of melon, carrot, and -susceptible and -resistant cotton, cowpea, pepper, and tomato for from California.

The host status of carrot, melon, and susceptible and resistant cultivars of tomato, cotton, cowpea, and pepper for a California isolate of the peach root-knot nematode was determined in greenhouse pot experiments. It was compared to a race 3 isolate of . Melon was an excellent host for both isolates and roots were heavily galled after the 8-week trial.

Establishing how much improvement in lung function and distance walked is clinically important for adult patients with Pompe disease.

Background And Purpose: Pompe disease is a rare, inheritable, progressive metabolic myopathy. This study aimed to estimate the minimal clinically important difference (MCID) for an improvement in forced vital capacity in the upright seated position (FVC) and the 6-min walk test (6MWT) after a year of treatment with enzyme replacement therapy.

Methods: Data were obtained from two prospective follow-up studies.

Lentiviral gene therapy with IGF2-tagged GAA normalizes the skeletal muscle proteome in murine Pompe disease.

Pompe disease is a lysosomal storage disorder caused by deficiency of acid alpha-glucosidase (GAA), resulting in glycogen accumulation with profound pathology in skeletal muscle. We recently developed an optimized form of lentiviral gene therapy for Pompe disease in which a codon-optimized version of the GAA transgene (LV-GAAco) was fused to an insulin-like growth factor 2 (IGF2) peptide (LV-IGF2.GAAco), to promote cellular uptake via the cation-independent mannose-6-phosphate/IGF2 receptor.

Twenty-Three-Year-Old Patient With Chronic Pain and Fainting Spells-A Clinical Vignette.

Care for and clinical presentation of patients with connective tissue disorders, specifically hypermobile Ehlers-Danlos syndrome, is poorly understood. Diagnosis can often take years, and comprehensive care can be difficult to coordinate for these patients. This vignette aims to show the clinical characteristics of a young female with Hypermobile Ehlers-Danlos, as well as the evaluation of her diagnostic presentation and subsequent treatment.

Neurofilament light protein as a biomarker for spinal muscular atrophy: a review and reference ranges.

Spinal muscular atrophy (SMA) is the leading genetic cause of infant mortality, characterized by progressive neuromuscular degeneration resulting from mutations in the survival motor neuron () gene. The availability of disease-modifying therapies for SMA therapies highlights the pressing need for easily accessible and cost-effective blood biomarkers to monitor treatment response and for better disease management. Additionally, the wide implementation of newborn genetic screening programs in Western countries enables presymptomatic diagnosis of SMA and immediate treatment administration.

A Comparison Between and from California on Susceptible and Resistant Sweetpotato Cultivars.

The reproduction and ability to cause root-galling of a California isolate of the peach root-knot nematode was evaluated on seven sweetpotato () cultivars and compared with an race 3 and an -gene resistance-breaking isolate. The susceptible tomato () cultivar Daniela and the -gene-carrying resistant cultivar Celebrity were included as controls. Repeated trials were done in pots in a nematode-quarantine greenhouse at the University of California, Riverside.

Lentiviral Gene Therapy for Mucopolysaccharidosis II with Tagged Iduronate 2-Sulfatase Prevents Life-Threatening Pathology in Peripheral Tissues But Fails to Correct Cartilage.

Deficiency of iduronate 2-sulfatase (IDS) causes Mucopolysaccharidosis type II (MPS II), a lysosomal storage disorder characterized by systemic accumulation of glycosaminoglycans (GAGs), leading to a devastating cognitive decline and life-threatening respiratory and cardiac complications. We previously found that hematopoietic stem and progenitor cell-mediated lentiviral gene therapy (HSPC-LVGT) employing tagged IDS with insulin-like growth factor 2 (IGF2) or ApoE2, but not receptor-associated protein minimal peptide (RAP12x2), efficiently prevented brain pathology in a murine model of MPS II. In this study, we report on the effects of HSPC-LVGT on peripheral pathology and we analyzed IDS biodistribution.

Tagged IDS causes efficient and engraftment-independent prevention of brain pathology during lentiviral gene therapy for Mucopolysaccharidosis type II.

Mucopolysaccharidosis type II (OMIM 309900) is a lysosomal storage disorder caused by iduronate 2-sulfatase (IDS) deficiency and accumulation of glycosaminoglycans, leading to progressive neurodegeneration. As intravenously infused enzyme replacement therapy cannot cross the blood-brain barrier (BBB), it fails to treat brain pathology, highlighting the unmet medical need to develop alternative therapies. Here, we test modified versions of hematopoietic stem and progenitor cell (HSPC)-mediated lentiviral gene therapy (LVGT) using IDS tagging in combination with the ubiquitous MND promoter to optimize efficacy in brain and to investigate its mechanism of action.

The impact of COVID-19 infection, the pandemic and its associated control measures on patients with Pompe disease.

Background: Patients with Pompe disease, a rare metabolic myopathy, were thought to be at increased risk of severe COVID-19 disease during the pandemic. In addition, the lockdown may have affected their regular treatment.

Objective: To assess the perceived effect of COVID-19 infection and of the pandemic on the treatment, and physical and mental health of patients with Pompe disease.

Tomato -gene Resistance-Breaking Populations of Show Variable Reproduction on Susceptible and Resistant Crop Cultivars.

Sixteen isolates from tomato fields in California grown with resistant cultivars were multiplied on resistant tomato in a greenhouse. Of these resistance-breaking isolates, one was identified as , and all others as The reproduction of the isolate and four isolates on six resistant tomato cultivars and on susceptible and resistant cultivars of pepper, sweetpotato, green bean, cotton, and cowpea was evaluated and compared to an avirulent population in greenhouse pot trials. On resistant tomato cultivars, there were minor but significant differences between the resistance-breaking isolates and between the different tomato cultivars.

Long-term benefits of physical activity in adult patients with late onset Pompe disease: a retrospective cohort study with 10 years of follow-up.

Background: In 2011 a 12 weeks personalized exercise training program in 23 mildly affected adult late onset Pompe patients (age 19.6-70.5 years) improved endurance, muscle strength and function.