| scholarly article | Q13442814 |
| P356 | DOI | 10.1002/JNR.21134 |
| P698 | PubMed publication ID | 17139681 |
| P2093 | author name string | Paola Di Natale | |
| Raffaella Faraonio | |||
| Guglielmo R D Villani | |||
| Sigismondo Castaldo | |||
| Enrico Gonzalez Y Reyero | |||
| Nadia Gargiulo | |||
| P2860 | cites work | Molecular basis of programmed cell death involved in neurodegeneration | Q36282417 |
| Monocyte chemoattractant protein-1 and CCR2 interactions are required for IFN-alpha/beta-induced inflammatory responses and antiviral defense in liver | Q45232017 | ||
| P433 | issue | 3 | |
| P304 | page(s) | 612-622 | |
| P577 | publication date | 2007-02-01 | |
| P1433 | published in | Journal of Neuroscience Research | Q6295654 |
| P1476 | title | Cytokines, neurotrophins, and oxidative stress in brain disease from mucopolysaccharidosis IIIB. | |
| P478 | volume | 85 |
| Q36521756 | A GLP-Compliant Toxicology and Biodistribution Study: Systemic Delivery of an rAAV9 Vector for the Treatment of Mucopolysaccharidosis IIIB. |
| Q45881431 | A GLP-compliant toxicology and biodistribution study: systemic delivery of a rAAV9 vector for the treatment of mucopolysaccharidosis IIIB. |
| Q50693771 | A novel conditional Sgsh knockout mouse model recapitulates phenotypic and neuropathic deficits of Sanfilippo syndrome |
| Q38300203 | Activation of stress kinases in the brain of mucopolysaccharidosis IIIB mice |
| Q46182790 | Alterations in oxidative markers in the cerebellum and peripheral organs in MPS I mice |
| Q35053456 | Amyloidosis, synucleinopathy, and prion encephalopathy in a neuropathic lysosomal storage disease: the CNS-biomarker potential of peripheral blood |
| Q91140910 | Anatomical changes and pathophysiology of the brain in mucopolysaccharidosis disorders |
| Q42176546 | Biochemical, histological and functional correction of mucopolysaccharidosis type IIIB by intra-cerebrospinal fluid gene therapy |
| Q55658045 | Brain-targeted stem cell gene therapy corrects mucopolysaccharidosis type II via multiple mechanisms. |
| Q33692991 | Cell- and gene-based therapeutic approaches for neurological deficits in mucopolysaccharidoses |
| Q34982831 | Combination therapies for lysosomal storage disease: is the whole greater than the sum of its parts? |
| Q42059455 | Correction of Neurological Disease of Mucopolysaccharidosis IIIB in Adult Mice by rAAV9 Trans-Blood–Brain Barrier Gene Delivery |
| Q63197179 | DNA damage in leukocytes from pretreatment mucopolysaccharidosis type II patients; protective effect of enzyme replacement therapy |
| Q34077919 | Defects in the Medial Entorhinal Cortex and Dentate Gyrus in the Mouse Model of Sanfilippo Syndrome Type B |
| Q36433328 | Differential Expression of Matrix Metalloproteinases in the Serum of Patients with Mucopolysaccharidoses |
| Q34757062 | Differential distribution of heparan sulfate glycoforms and elevated expression of heparan sulfate biosynthetic enzyme genes in the brain of mucopolysaccharidosis IIIB mice |
| Q35559945 | Direct gene transfer to the CNS prevents emergence of neurologic disease in a murine model of mucopolysaccharidosis type I |
| Q40277701 | Disease correction by AAV-mediated gene therapy in a new mouse model of mucopolysaccharidosis type IIID. |
| Q28472880 | Early neurodegeneration progresses independently of microglial activation by heparan sulfate in the brain of mucopolysaccharidosis IIIB mice |
| Q36322040 | Electrophysiological and Histological Characterization of Rod-Cone Retinal Degeneration and Microglia Activation in a Mouse Model of Mucopolysaccharidosis Type IIIB. |
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| Q37987877 | Gene expression-targeted isoflavone therapy |
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| Q35108104 | Glycosaminoglycan storage in neuroanatomical regions of mucopolysaccharidosis I dogs following intrathecal recombinant human iduronidase |
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| Q37739706 | Lysosomal storage disease: revealing lysosomal function and physiology. |
| Q41838643 | Mannitol-facilitated CNS entry of rAAV2 vector significantly delayed the neurological disease progression in MPS IIIB mice |
| Q89509285 | Molecular Bases of Neurodegeneration and Cognitive Decline, the Major Burden of Sanfilippo Disease |
| Q38105045 | Mucopolysaccharide diseases: a complex interplay between neuroinflammation, microglial activation and adaptive immunity. |
| Q30495848 | Mucopolysaccharidosis IIIB, a lysosomal storage disease, triggers a pathogenic CNS autoimmune response |
| Q38004963 | Mucopolysaccharidosis type I: current knowledge on its pathophysiological mechanisms |
| Q36893989 | Mucopolysaccharidosis type IIIB (MPS IIIB) masquerading as a behavioural disorder |
| Q48799845 | N-butyldeoxynojirimycin treatment restores the innate fear response and improves learning in mucopolysaccharidosis IIIA mice. |
| Q38111373 | NADPH Oxidase and Neurodegeneration |
| Q38988296 | Near-Complete Correction of Profound Metabolomic Impairments Corresponding to Functional Benefit in MPS IIIB Mice after IV rAAV9-hNAGLU Gene Delivery |
| Q59340086 | Neurobehavioral phenotypes of neuronopathic mucopolysaccharidoses |
| Q27303930 | Neuroinflammation, mitochondrial defects and neurodegeneration in mucopolysaccharidosis III type C mouse model. |
| Q42748597 | Neuroinflammatory and oxidative stress phenomena in MPS IIIA mouse model: The positive effect of long-term aspirin treatment |
| Q36232187 | Neuroinflammatory paradigms in lysosomal storage diseases |
| Q37212850 | Neurological findings in Hunter disease: pathology and possible therapeutic effects reviewed |
| Q55387411 | Neuromelanin organelles are specialized autolysosomes that accumulate undegraded proteins and lipids in aging human brain and are likely involved in Parkinson's disease. |
| Q38415235 | Novel grooved substrata stimulate macrophage fusion, CCL2 and MMP-9 secretion |
| Q63197185 | Oxidative stress in patients with mucopolysaccharidosis type II before and during enzyme replacement therapy |
| Q46850843 | Pathological and biochemical studies of mucopolysaccharidosis type IIIB (Sanfilippo syndrome type B) in juvenile emus (Dromaius novaehollandiae). |
| Q37739318 | Pathophysiology of neuropathic lysosomal storage disorders |
| Q34438623 | Peripheral Nervous System Neuropathology and Progressive Sensory Impairments in a Mouse Model of Mucopolysaccharidosis IIIB |
| Q37305882 | Progressive neurologic and somatic disease in a novel mouse model of human mucopolysaccharidosis type IIIC. |
| Q41614431 | Repeated administrations of human umbilical cord blood cells improve disease outcomes in a mouse model of Sanfilippo syndrome type III B. |
| Q33439351 | Sanfilippo syndrome type B, a lysosomal storage disease, is also a tauopathy |
| Q37130107 | Sanfilippo syndrome: a mini-review |
| Q45857027 | Serum MIP-1 alpha level: a biomarker for the follow-up of lentiviral therapy in mucopolysaccharidosis IIIB mice |
| Q38851110 | Serum global metabolomics profiling reveals profound metabolic impairments in patients with MPS IIIA and MPS IIIB. |
| Q93206516 | The role of innate immunity in mucopolysaccharide diseases |
| Q36516376 | Two-year follow-up of Sanfilippo Disease patients treated with a genistein-rich isoflavone extract: assessment of effects on cognitive functions and general status of patients |
| Q48835669 | Unfolded protein response is not activated in the mucopolysaccharidoses but protein disulfide isomerase 5 is deregulated. |
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