The scope of the crustacean immune system for disease control

The culture or wild capture of marine and freshwater shellfish, including crustaceans, is without doubt a key source of protein for a burgeoning world population. Historically the expansion of aquaculture has, however, been accompanied by the increased incidence of economically significant diseases, most notably of viral and bacterial origin. Since the late 1970s great progress has been made in our understanding of the generalized protostome innate immune system. Distinct pathways, pathogen receptor proteins and effector molecules have since been identified that are not ancestral or homologous to those of the deuterostomes, including vertebrates. Within the past decade progress has accelerated with the rapid characterisation of new classes of recognition proteins, immune effectors and regulatory pathways. This paper provides a broad overview of our current understanding of invertebrate immunology, taking the crustacean decapod immune system as its focus. Recent developments in the field are described briefly and their implications and potential considered. These advances offer fundamental new insights in our efforts to understand disease in cultured populations and also to develop knowledge of environmental effects on host/pathogen interactions within a fishery context. Of course, challenges do remain, including the lack of an immortal cell line and the limited publically-available genomic resources. These are considered in this review as priorities for future research effort. With the continued application of more insightful technologies, coupled with associated investment, it is expected that the speed at which some of these issues are resolved will accelerate

Dysfunction of Heterotrimeric Kinesin-2 in Rod Photoreceptor Cells and the Role of Opsin Mislocalization in Rapid Cell Death

Loss of kinesin-2 function causes rapid death of rod photoreceptors. The cell death is dependent on the expression of opsin, which first accumulates along the route to the outer segment, but not on signaling by opsin-arrestin complexes or by light activation; the key element appears to be the accumulation of excessive protein in the wrong place

Cataract Surgery with Foldable Single Piece IOLs in Congenital Cataract-Microcornea Syndrome

Objective: To present the visual outcomes and intraocular lens (IOLs) stability after cataract surgery with foldable single piece IOLs in a patient with congenital cataract-microcornea syndrome (CCMC). Case presentation: A 28-year-old woman presented with bilateral microcornea and posterior polar cataract. Her uncorrected (UDVA) and corrected (CDVA) distance visual acuities were 20/100, 20/160 and 20/80, 20/80, respectively. The horizontal cornea diameter was 9.0 mm in both eyes. Anterior chamber angle assessment (ACAA) with Scheie classification showed grade 3 on the superior, inferior, and temporal site and grade 2 on the nasal site in both eyes. On ultrasound biomicroscopy (UBM), the anterior chamber depths were 2.35 mm. OD and 2.41 mm. OS. The axial lengths were 23.28 mm., OD and 22.50 mm. OS with the A-scan ultrasound biometry. The IOLs power calculation with SRK/T formulas was +25.00 diopter OD and +22.50 diopter OS (A-constant 118.4). Phacoemulsification was performed on both eyes. After lens aspiration, ruptured posterior capsule, a common complication occurred. Then anterior vitrectomy was performed. A foldable single piece IOLs without haptic trimming was implanted in the sulcus in each eye. CDVA was 20/63 equally in both eyes. The intraocular pressure was 12 and 14 mmHg. ACAA showed grade 2 and no pigment dispersion. The IOLs had no tilt or decentration. UBM showed the IOLs were in the proper position and were not rubbing the iris. Conclusion: The foldable single piece IOLs without haptic trimming in the sulcus were safely implanted in both eyes with microcornea in CCMC

Function of MYO7A in the Human RPE and the Validity of Shaker1 Mice as a Model for Usher Syndrome 1B

Phagosome digestion and melanosome motility were studied in human primary RPE cells. RNAi knockdown studies showed that MYO7A functions to constrain rapid, long-range movements of melanosomes. This function is comparable to that in mouse RPE, supporting the use of MYO7A-null mice and the correction of mutant phenotypes in their RPE as an outcome measure in preclinical studies for therapies of Usher syndrome type 1B

Harmonin (Ush1c) is required in zebrafish Muller glial cells for photoreceptor synaptic development and function

Usher syndrome is the most prevalent cause of hereditary deaf-blindness, characterized by congenital sensorineural hearing impairment and progressive photoreceptor degeneration beginning in childhood or adolescence. Diagnosis and management of this disease are complex, and the molecular changes underlying sensory cell impairment remain poorly understood. Here we characterize two zebrafish models for a severe form of Usher syndrome, Usher syndrome type 1C (USH1C): one model is a mutant with a newly identified ush1c nonsense mutation, and the other is a morpholino knockdown of ush1c. Both have defects in hearing, balance and visual function from the first week of life. Histological analyses reveal specific defects in sensory cell structure that are consistent with these behavioral phenotypes and could implicate Müller glia in the retinal pathology of Usher syndrome. This study shows that visual defects associated with loss of ush1c function in zebrafish can be detected from the onset of vision, and thus could be applicable to early diagnosis for USH1C patients