Ocular changes in some types of the mucopolysaccharidoses (MPS) include corneal clouding, glaucoma, pigmentary retinopathy, optic disc edema, and optic atrophy. Pigmentary retinopathy has been reported in MPS I (Hurler disease), MPS I-S (Scheie syndrome), MPS I-H/S (Hurler/Scheie), MPS II (Hunter disease), MPS III (Sanfilippo syndrome), and MPS IV A (Morquio A syndrome). Clinical signs of retin...

Sanfilippo syndrome, or mucopolysaccharidosis (MPS) type III, refers to one of five autosomal recessive, neurodegenerative lysosomal storage disorders (MPS IIIA to MPS IIIE) whose symptoms are caused by the deficiency of enzymes involved exclusively in heparan sulfate degradation. The primary characteristic of MPS III is the degeneration of the central nervous system, resulting in mental retard...

BACKGROUND Microparticles (MPs) are circulating membrane particles of less than a micrometer in diameter shed from endothelial and blood cells. Recent literature suggests that MPs are not just functionally inert cell debris but may possess biological functions and mediate the communication between vascular cells. As a significant proportion of MPs originate from platelets and endothelial cells,...

Massively parallel sequencing (MPS) is rapidly evolving and is starting to be utilized by the clinical field as well as diagnostics. We describe major recent advances that have come about as a result of the application of MPS in the biomedical field and the first approaches in medical genetics that have made use of MPS. Without any doubt, MPS has proven to be a very powerful technique. To unrav...

Severe cardiac involvement is a common feature of mucopolysaccharidoses (MPS), but occurs only rarely in MPS III (Sanfilippo syndrome). We report herein a case of MPS III-A having cardiac involvement as its first manifestation. Analysis of the SGSH gene showed homozygosity for the novel mutation p.G80V. We propose that MPS disorders, including MPS III-A, should be included in the differential d...

Keratan sulfate (KS) is commonly elevated in urine samples from patients with mucopolysaccharidosis type IVA (MPS IVA) and is considered pathognomonic for the condition. Recently, a new method has been described by Martell et al. to detect and measure urinary KS utilizing LC-MS/MS. As a part of the validation of this method in our laboratory, we studied the sensitivity and specificity of elevat...

BACKGROUND Microparticles (MPs) are vesicles released from plasma membrane upon cell activation and during apoptosis. Human T lymphocytes undergoing activation and apoptosis generate MPs bearing morphogen Shh (MPs(Shh+)) that are able to regulate in vitro angiogenesis. METHODOLOGY/PRINCIPAL FINDINGS Here, we investigated the ability of MPs(Shh+) to modulate neovascularization in a model of mo...

MPS VI1 mice are deficient in P-glucuronidase and share many clinical, biochemical, and pathologic characteristics with human mucopolysaccharidosis type VI1 (MPS VII). We have shown that syngeneic bone marrow transplantation (BMT) prolongs survival and reduces lysosomal storage in many organs of the MPS VI1 mouse. In this report, we quantify the hearing loss and determine the impact of syngenei...

Abstract. A strategic placement of mesh points (MPs) in a Wireless Mesh Network (WMN) is essential to maximize the throughput of the network. In this paper, we address the problem of MPs placement for throughput optimization in WMN with consideration of routing protocols. Specifically, we formulate the routing paths among the MPs and mesh routers (MRs), calculate the aggregated traffic at the M...

Mononuclear phagocytes (MPs) from human peripheral blood were separated on discontinuous Percoll gradients into two subpopulations. A high density population was isolated which contained less mature MPs, while the MPs in the low density population were more mature cells. The proportion of high density MPs was found to increase sharply in conjunction with the peripheral blood monocytosis associa...