Longitudinal structure-function analysis of molecularly-confirmed CYP4V2 Bietti Crystalline Dystrophy.

Objectives: Bietti Crystalline Dystrophy (BCD) is an autosomal recessive progressive retinal disease caused by mutations in CYP4V2. We have characterised the natural history including structural and functional measures to identify potential outcome metrics for future clinical trials.

Methods: Molecularly-confirmed BCD patients with biallelic variants in CYP4V2 were retrospectively identified from Moorfields Eye Hospital (UK).

Osteocyte-Derived CaMKK2 Regulates Osteoclasts and Bone Mass in a Sex-Dependent Manner through Secreted Calpastatin.

Calcium/calmodulin (CaM)-dependent protein kinase kinase 2 (CaMKK2) regulates bone remodeling through its effects on osteoblasts and osteoclasts. However, its role in osteocytes, the most abundant bone cell type and the master regulator of bone remodeling, remains unknown. Here we report that the conditional deletion of CaMKK2 from osteocytes using Dentine matrix protein 1 ()-8kb- mice led to enhanced bone mass only in female mice owing to a suppression of osteoclasts.

Review of updated regulations and product license categories for veterinary vaccines in the United States.

The USDA continues to consider and implement regulatory pathways for evolving scenarios, needs, and technologies. The intent of this report is to make veterinarians and other users of veterinary biologics aware of recent regulatory additions and changes, particularly in the area of veterinary vaccines. These include new licensure pathways to increase product availability, standardization of labeling, and increased transparency regarding adverse event reports and the efficacy and safety studies accepted by the USDA for product licensure.

Translating research into licensed vaccines and validated and licensed diagnostic tests.

The USDA Center for Veterinary Biologics (CVB) has the regulatory authority to issue licenses and permits that allow the marketing of pure, safe, potent, and effective veterinary biological products. Under the standard licensing or permitting process, a manufacturer develops, characterizes, and evaluates a product prior to licensure. The CVB evaluates the submitted information, inspects the manufacturing facilities and methods of production and testing, and confirms key product test results through independent testing.

Abi1 gene silencing by short hairpin RNA impairs Bcr-Abl-induced cell adhesion and migration in vitro and leukemogenesis in vivo.

Abl interactor (Abi) 1 was first identified as the downstream target of Abl tyrosine kinases and was found to be dysregulated in leukemic cells expressing oncogenic Bcr-Abl and v-Abl. Although the accumulating evidence supports a role of Abi1 in actin cytoskeleton remodeling and growth factor/receptor signaling, it is not clear how it contributes to Bcr-Abl-induced leukemogenesis. We show here that Abi1 gene silencing by short hairpin RNA attenuated the Bcr-Abl-induced abnormal actin remodeling, membrane-type 1 metalloproteinase clustering and inhibited cell adhesion and migration on fibronectin-coated surfaces.

Bcr-Abl induces abnormal cytoskeleton remodeling, beta1 integrin clustering and increased cell adhesion to fibronectin through the Abl interactor 1 pathway.

Hematopoietic cells isolated from patients with Bcr-Abl-positive leukemia exhibit multiple abnormalities of cytoskeletal and integrin function. These abnormalities are thought to play a role in the pathogenesis of leukemia; however, the molecular events leading to these abnormalities are not fully understood. We show here that the Abi1 pathway is required for Bcr-Abl to stimulate actin cytoskeleton remodeling, integrin clustering and cell adhesion.

Prognostic analysis of pre-transplant peripheral T-cell levels in patients receiving an autologous hematopoietic progenitor-cell transplant.

The purpose of this study was to evaluate pre-transplant T-cell status in autologous hematopoietic progenitor-cell transplantation (HPCT) recipients. Between 1999 and 2002 we prospectively enrolled 85 autologous HPCT recipients with solid tumors (N = 50) or hematological malignancies (n = 35). Patient diagnoses included breast cancer (N = 49), non-Hodgkin's lymphoma (N = 20), myeloma (N = 11), Hodgkin's disease (N = 3), germ-cell tumor (N = 1) and amyloidosis (N = 1).

Development of monoclonal antibodies suitable for use in antigen quantification potency tests for clostridial veterinary vaccines.

The quality control testing of clostridial veterinary vaccines currently requires large numbers of animals. Alternative in vitro test methods are being investigated by researchers in industry and by regulatory authorities in many countries. Monoclonal antibodies that neutralize Clostridium perfringens alpha toxin, C.

Quantitation of commercial equine tetanus antitoxin by competitive enzyme-linked immunosorbent assay.

In the USA, the potency of commercially prepared equine tetanus antitoxin is determined by the method outlined in the Code of Federal Regulations, Title 9, Part 113.451. In the current test, commercial equine tetanus antitoxin is tested by a toxin neutralization test in guinea pigs.

The cost of pressure area management in an intensive care unit.

The aim of the study was to determine the cost of prevention and treatment of pressure sores in an intensive care unit. We studied prospectively the cost of pressure area management for one year from April 1, 1990, collecting data on 2482 patient days. The 638 patients were divided into the 'prevention group' (525 patients), who did not have or develop pressure-related tissue injury, and the 'therapy group' (113 patients), who were admitted with or developed a sore.