Pharmacokinetic and Pharmacodynamic Modeling to Optimize the Dose of Vestronidase Alfa, an Enzyme Replacement Therapy for Treatment of Patients with Mucopolysaccharidosis Type VII: Results from Three Trials
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DOI: 10.1007/s40262-018-0721-y
Abstract: IntroductionMucopolysaccharidosis type VII (MPS VII, Sly Syndrome) is a progressive, debilitating, ultra-rare lysosomal storage disorder caused by the deficiency of β-glucuronidase (GUS), an enzyme required for breakdown of glycosaminoglycans (GAGs). Vestronidase alfa, a recombinant human… read more here.
Keywords: treatment; vii; type vii; mps vii ... See more keywords
Molecular Basis for Immunity Protein Recognition of a Type VII Secretion System Exported Antibacterial Toxin
Sign Up to like & getrecommendations! Published in 2018 at "Journal of Molecular Biology"
DOI: 10.1016/j.jmb.2018.08.027
Abstract: Gram-positive bacteria deploy the type VII secretion system (T7SS) to facilitate interactions between eukaryotic and prokaryotic cells. In recent work, we identified the TelC protein from Streptococcus intermedius as a T7SS-exported lipid II phosphatase that… read more here.
Keywords: vii secretion; secretion system; telc; immunity protein ... See more keywords
Bacterial secretion chaperones: the mycobacterial type VII case
Sign Up to like & getrecommendations! Published in 2018 at "FEMS Microbiology Letters"
DOI: 10.1093/femsle/fny197
Abstract: Abstract Chaperones are central players in maintaining the proteostasis in all living cells. Besides highly conserved generic chaperones that assist protein folding and assembly in the cytosol, additional more specific chaperones have evolved to ensure… read more here.
Keywords: mycobacterial type; type vii; secretion; secretion chaperones ... See more keywords
EssC is a specificity determinant for Staphylococcus aureus type VII secretion
Sign Up to like & getrecommendations! Published in 2018 at "Microbiology"
DOI: 10.1099/mic.0.000650
Abstract: The type VII protein secretion system (T7SS) is found in actinobacteria and firmicutes, and plays important roles in virulence and interbacterial competition. A membrane-bound ATPase protein, EssC in Staphylococcus aureus, lies at the heart of… read more here.
Keywords: protein; staphylococcus aureus; secretion; essc specificity ... See more keywords
Structural and functional characteristics of the tripartite ABC transporter.
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DOI: 10.1099/mic.0.001257
Abstract: ATP-binding cassette (ABC) transporters are one of the largest protein superfamilies and are found in all living organisms. These transporters use the energy from ATP binding and hydrolysis to transport various substrates. In this review,… read more here.
Keywords: abc transporters; vii abc; type vii; abc ... See more keywords
Unique mouse monoclonal antibodies reactive with maturation‐related epitopes on type VII collagen
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DOI: 10.1111/exd.13306
Abstract: In this study, we generated a new set of monoclonal antibodies (mAbs) to bovine and human type VII collagen (COL7) by immunizing mice with bovine cornea‐derived basement membrane zone (BMZ) fraction. The four mAbs, tentatively… read more here.
Keywords: collagen; monoclonal antibodies; col7; col7 like ... See more keywords
Genes and compounds that increase type VII collagen expression as potential treatments for dystrophic epidermolysis bullosa
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DOI: 10.1111/exd.14555
Abstract: Dystrophic epidermolysis bullosa (DEB) is a skin‐blistering disease caused by mutations in COL7A1, which encodes type VII collagen (C7). There is no cure for DEB, but previous work has shown potential therapeutic benefit of increased… read more here.
Keywords: vii collagen; epidermolysis bullosa; compounds increase; expression ... See more keywords
Tolerance induction and microglial engraftment after fetal therapy without conditioning in mice with mucopolysaccharidosis type VII
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DOI: 10.1126/scitranslmed.aay8980
Abstract: In utero therapy with recombinant enzyme or hematopoietic stem cells in MPS7 mice induces tolerance to missing enzyme and corrects brain microglia. When treating at birth is too late Mucopolysaccharidosis type VII (MPS7) is a… read more here.
Keywords: mucopolysaccharidosis type; mps7; type vii; mice ... See more keywords
The Antibacterial Type VII Secretion System of Bacillus subtilis: Structure and Interactions of the Pseudokinase YukC/EssB
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DOI: 10.1128/mbio.00134-22
Abstract: Type VII secretion systems mediate protein extrusion from Gram-positive bacteria and are classified as T7SSa and T7SSb in Actinobacteria and in Firmicutes, respectively. Despite the genetic divergence of T7SSa and T7SSb, the high degree of… read more here.
Keywords: structure; t7ssb; secretion; type vii ... See more keywords
Infect and Inject: How Mycobacterium tuberculosis Exploits Its Major Virulence-Associated Type VII Secretion System, ESX-1.
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DOI: 10.1128/microbiolspec.bai-0024-2019
Abstract: Mycobacterium tuberculosis is an ancient master of the art of causing human disease. One important weapon within its fully loaded arsenal is the type VII secretion system. M. tuberculosis has five of them: ESAT-6 secretion… read more here.
Keywords: mycobacterium tuberculosis; vii secretion; type vii; tuberculosis ... See more keywords
Flotillin scaffold activity contributes to type VII secretion system assembly in Staphylococcus aureus
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DOI: 10.1371/journal.ppat.1006728
Abstract: Scaffold proteins are ubiquitous chaperones that promote efficient interactions between partners of multi-enzymatic protein complexes; although they are well studied in eukaryotes, their role in prokaryotic systems is poorly understood. Bacterial membranes have functional membrane… read more here.
Keywords: vii secretion; staphylococcus aureus; scaffold activity; type vii ... See more keywords