Search Results - "Garman, Scott C."

CaMKII binds both substrates and activators at the active site by Özden, Can, Sloutsky, Roman, Mitsugi, Tomohiro, Santos, Nicholas, Agnello, Emily, Gaubitz, Christl, Foster, Joshua, Lapinskas, Emily, Esposito, Edward A., Saneyoshi, Takeo, Kelch, Brian A., Garman, Scott C., Hayashi, Yasunori, Stratton, Margaret M.

“…Ca2+/calmodulin-dependent protein kinase II (CaMKII) is a signaling protein required for long-term memory. When activated by Ca2+/CaM, it sustains activity…”
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Human acid sphingomyelinase structures provide insight to molecular basis of Niemann–Pick disease by Zhou, Yan-Feng, Metcalf, Matthew C., Garman, Scott C., Edmunds, Tim, Qiu, Huawei, Wei, Ronnie R.

“…Acid sphingomyelinase (ASM) hydrolyzes sphingomyelin to ceramide and phosphocholine, essential components of myelin in neurons. Genetic alterations in ASM lead…”
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The glycan code of the endoplasmic reticulum: asparagine-linked carbohydrates as protein maturation and quality-control tags by Hebert, Daniel N., Garman, Scott C., Molinari, Maurizio

“…The majority of proteins that traverse the secretory pathway receive asparagine (Asn)-linked glycosylations. Glycans are bulky hydrophilic modifications that…”
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Pharmacological chaperones for human α-N-acetylgalactosaminidase by Clark, Nathaniel E, Metcalf, Matthew C, Best, Daniel, Fleet, George W. J, Garman, Scott C

“…Schindler/Kanzaki disease is an inherited metabolic disease with no current treatment options. This neurologic disease results from a defect in the lysosomal…”
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Mutant alpha-galactosidase A enzymes identified in Fabry disease patients with residual enzyme activity: biochemical characterization and restoration of normal intracellular processing by 1-deoxygalactonojirimycin by Ishii, Satoshi, Chang, Hui-Hwa, Kawasaki, Kunito, Yasuda, Kayo, Wu, Hui-Li, Garman, Scott C, Fan, Jian-Qiang

“…Fabry disease is a lysosomal storage disorder caused by the deficiency of alpha-Gal A (alpha-galactosidase A) activity. In order to understand the molecular…”
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Adaptor-Dependent Degradation of a Cell-Cycle Regulator Uses a Unique Substrate Architecture by Rood, Keith L., Clark, Nathaniel E., Stoddard, Patrick R., Garman, Scott C., Chien, Peter

“…In Caulobacter crescentus, the ClpXP protease degrades several crucial cell-cycle regulators, including the phosphodiesterase PdeA. Degradation of PdeA…”
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Pediatric Fabry Disease by Ries, Markus, Gupta, Surya, Moore, David F, Sachdev, Vandana, Quirk, Jane M, Murray, Gary J, Rosing, Douglas R, Robinson, Chevalia, Schaefer, Ellen, Gal, Andreas, Dambrosia, James M, Garman, Scott C, Brady, Roscoe O, Schiffmann, Raphael

“…Fabry disease is an underdiagnosed, treatable, X-linked, multisystem disorder. To test the hypothesis that quality of life and sweating are decreased among…”
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The Structure of Human GALNS Reveals the Molecular Basis for Mucopolysaccharidosis IV A by Rivera-Colón, Yadilette, Schutsky, Emily K., Kita, Adriana Z., Garman, Scott C.

“…Lysosomal enzymes catalyze the breakdown of macromolecules in the cell. In humans, loss of activity of a lysosomal enzyme leads to an inherited metabolic…”
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Proteolytic Activation of Human Cathepsin A by Kolli, Nilima, Garman, Scott C.

“…Galactosialidosis is a human lysosomal storage disease caused by deficiency in the multifunctional lysosomal protease cathepsin A (also known as protective…”
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Ca2+/CaM dependent protein kinase II (CaMKII)α and CaMKIIβ hub domains adopt distinct oligomeric states and stabilities by Özden, Can, MacManus, Sara, Adafia, Ruth, Samkutty, Alfred, Torres‐Ocampo, Ana P., Garman, Scott C., Stratton, Margaret M.

“…Ca2+/calmodulin‐dependent protein kinase II (CaMKII) is a multidomain serine/threonine kinase that plays important roles in the brain, heart, muscle tissue,…”
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Unlocking a Caged Lysosomal Protein from a Polymeric Nanogel with a pH Trigger by Molla, Mijanur Rahaman, Marcinko, Tyler, Prasad, Priyaa, Deming, Derrick, Garman, Scott C, Thayumanavan, S

“…A polymeric nanogel has been used to sequester and turn off a lysosomal protein, acid α-glucosidase (GAA). The nanogel contains a β-thiopropionate…”
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Characterization of CaMKIIα holoenzyme stability by Torres‐Ocampo, Ana P., Özden, Can, Hommer, Alexandra, Gardella, Anne, Lapinskas, Emily, Samkutty, Alfred, Esposito, Edward, Garman, Scott C, Stratton, Margaret M

“…Ca2+/calmodulin‐dependent protein kinase II (CaMKII) is a Ser/Thr kinase necessary for long‐term memory formation and other Ca2+‐dependent signaling cascades…”
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The Molecular Defect Leading to Fabry Disease: Structure of Human α-Galactosidase by Garman, Scott C., Garboczi, David N.

“…Fabry disease is an X-linked lysosomal storage disease afflicting 1 in 40,000 males with chronic pain, vascular degeneration, cardiac impairment, and other…”
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Structure-function relationships in α-galactosidase A by Garman, Scott C

“…With recent interest in the molecular mechanisms responsible for Fabry disease, the number of known mutations in the GLA gene which encodes α‐galactosidase A…”
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The 1.9 Å Structure of Human α- N-Acetylgalactosaminidase: The Molecular Basis of Schindler and Kanzaki Diseases by Clark, Nathaniel E., Garman, Scott C.

“…α- N-acetylgalactosaminidase (α-NAGAL; E.C. 3.2.1.49) is a lysosomal exoglycosidase that cleaves terminal α- N-acetylgalactosamine residues from glycopeptides…”
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Ligand-promoted protein folding by biased kinetic partitioning by Hingorani, Karan S, Metcalf, Matthew C, Deming, Derrick T, Garman, Scott C, Powers, Evan T, Gierasch, Lila M

“…Pharmacological chaperones improve folding of destabilized Escherichia coli dihydrofolate reductase (DHFR) and human disease-linked α-galactosidase A (α-GAL)…”
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Phenotypic characteristics of the p.Asn215Ser (p.N215S) GLA mutation in male and female patients with Fabry disease: A multicenter Fabry Registry study by Germain, Dominique P., Brand, Eva, Burlina, Alessandro, Cecchi, Franco, Garman, Scott C., Kempf, Judy, Laney, Dawn A., Linhart, Aleš, Maródi, László, Nicholls, Kathy, Ortiz, Alberto, Pieruzzi, Federico, Shankar, Suma P., Waldek, Stephen, Wanner, Christoph, Jovanovic, Ana

“…Background The p.Asn215Ser or p.N215S GLA variant has been associated with late‐onset cardiac variant of Fabry disease. Methods To expand on the scarce…”
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Catalytic mechanism of human alpha-galactosidase by Guce, Abigail I, Clark, Nathaniel E, Salgado, Eric N, Ivanen, Dina R, Kulminskaya, Anna A, Brumer, 3rd, Harry, Garman, Scott C

“…The enzyme alpha-galactosidase (alpha-GAL, also known as alpha-GAL A; E.C. 3.2.1.22) is responsible for the breakdown of alpha-galactosides in the lysosome…”
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Electrostatic origin of in vitro aggregation of human γ-crystallin by Mohr, Benjamin G, Dobson, Cassidy M, Garman, Scott C, Muthukumar, Murugappan

“…The proteins α-, β-, and γ-crystallins are the major components of the lens in the human eye. Using dynamic light scattering method, we have performed in vitro…”
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Structure of the C-terminal Domains of Merozoite Surface Protein-1 from Plasmodium knowlesi Reveals a Novel Histidine Binding Site by Garman, Scott C, Simcoke, William N, Stowers, Anthony W, Garboczi, David N

“…The protozoan parasite Plasmodium causes malaria, with hundreds of millions of cases recorded annually. Protection against malaria infection can be conferred…”
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