Results 91 to 100 of about 97,768,729 (189)

Anaerobic sulfatase maturase AslB from Escherichia coli activates human recombinant iduronate-2-sulfate sulfatase (IDS) and N -acetylgalactosamine-6-sulfate sulfatase (GALNS)

, 2017
Maturation of type I sulfatases requires the conversion of the cysteine (Cys) or serine (Ser) present in the active site to formylglycine (FGly). This activation represents a limiting step during the production of recombinant sulfatases in bacteria and ...
Bonilla Y.A., Alméciga-Díaz C.J., Espejo-Mojica A.J., Sánchez O.F., Gonzalez-Santos J., Tolosa-Díaz A.D., Pimentel L.N., Patiño J.D., Rodríguez-López A., Yahir Andres Bonilla, Andrés Dario Tolosa-Díaz, Carlos Javier Alméciga-Díaz, Juan D. Patiño, Alexander Rodríguez-López, Angela J. Espejo-Mojica, Oscar F. Sánchez, Janneth Gonzalez-Santos, Luisa Natalia Pimentel   +17 more
core   +1 more source

Bioevaluation Of Heparin Oligosaccharides In Correcting Iduronate-2-Sulfatase Deficiency

, 2020
Introduction Mucopolysaccharidoses Type II is caused by an X-linked mutation in IDS gene which produce an enzyme called iduronate 2-sulfatase (IDS) that is essential for the breakdown of glycosaminoglycans. Lack of IDS enzyme activity leads to the accumulation of heparan sulfate and dermatan sulfate within the lysosomes.
Nordin, Fatimah Diana Amin, Affandi Omar, Balqis Kamarudin, Julaina Abdul Jalil   +3 more
openaire   +2 more sources

Sulfatase gene distribution and sub-classification in Planctomycetes and related strains.

, 2016
(a) Number of sulfatase genes in various Planctomycetes and related strains. Only sulfatase genes encoding the active site and ≥350 amino acid residues were included. (b) Functional subclasses of sulfatases present in P1, P2, P3, R.
Matthew Zane (2576311), John W. Stiller (197378), Jerry Jenkins (172005), Kurt LaButti (114830), Arthur R. Grossman (29860), Konstantinos Mavromatis (13836), Matt Nolan (2576314), Jay W. Kim (2576308), Jane Grimwood (172021), Simon Prochnik (61665), Mansi Chovatia (454148), Susan H. Brawley (392364), Jeremy Schmutz (172027)   +12 more
core   +1 more source

Proportional relationship between idursulfase-IT dose and CSF I2S AUC_inf and C_max.

, 2015
AUCinf, area under the serum concentration-time curve extrapolated to infinity; Cmax, maximum observed concentration; CSF, cerebrospinal fluid; I2S, iduronate-2-sulfatase.
Hongsheng Xie (716868), Thomas G. McCauley (716871), Jou-Ku Chung (716869), Mary Ann Mascelli (716870)   +3 more
core   +1 more source

Challenges and opportunities with providing genetic testing and counseling for mucopolysaccharidosis type II in Kenya

Orphanet Journal of Rare Diseases
Background Limited or absent genetic counseling and testing resources in low- and medium-income countries lead to missed or late diagnoses for treatable metabolic conditions with irreversible complications. In some communities, misunderstanding about the
Lucy N. Wainaina Mungai, Charles Njeru, Allan Njoroge, Michuki Maina, Syokau Ilovi, Ruth W. Nduati, Dalton Wamalwa, Beatrice Odongkara, Danny E. Miller   +8 more
doaj   +1 more source

Human sulfatase transiently and functionally active expressed in E. coli K12 [PDF]

, 2010
The recombinant human iduronate 2-sulfate sulfatase (hrIDS) was transiently and functionally active expressed in E. coli K12. The enzyme activity (crude extract) at 100 ml and 400 ml oscillated between 0.25 and 10.58 nmol h-1 mg-1.
Pe\uf1a, Olga Yaneth Echeverri, Poutou-Pi\uf1ales, Ra\ufal A., Lareo, Leonardo, S\ue1enz, Homero, Avellaneda, Luis A. Barrera, Homero Sáenz, Sáenz,Homero, Ni\uf1o, Adriana Vanegas, Raúl A. Poutou-Piñales, Poutou-Piñales,Raúl A, Landázuri,Patricia, Leonardo Lareo, Lareo,Leonardo, Echeverri Peña,Olga Yaneth, Olga Yaneth Echeverri Peña, Patricia Landázuri, Luis A. Barrera Avellaneda, Vanegas Niño,Adriana, Adriana Vanegas Niño, Barrera Avellaneda,Luis A, Land\ue1zuri, Patricia   +20 more
core   +1 more source

Scleral Thickening as an Etiology for Optic Disc edema in Hunter Syndrome

, 2023
Hunter syndrome is a Type II mucopolysaccharidoses (MPS) metabolic disorder that is defined by a deficiency of iduronate-2-sulfatase, leading to a buildup of glycosaminoglycans (GAGs) and clinical features such as developmental delay and facial ...
Terisa Yiin; Nandini Gandhi; Yin Liu
core  

遺伝性ムコ多糖代謝異常症II型 (Hunter病) の分子生物学的研究 I) Intermediate form of mucopolysaccharidosis type II (Hunter disease) : a C^<1327> to T substitution in the iduronate sulfatase gene. II) Mucopolysaccharidosis type II (Hunter disease) : identification and characterization of eight point mutations in the iduronate-2-sulfatase gene in Japanese patients.

博士論文 (Doctoral dissertation)I) Intermediate form of mucopolysaccharidosis type II (Hunter disease) : a C^ to T substitution in the iduronate sulfatase gene.II) Mucopolysaccharidosis type II (Hunter disease) : identification and characterization of eight ...
祐川, 和子
core  

Mass spectroscopy results of proteins interacting exclusively with POC5^A429V.

, 2019
Five proteins were found to be interacting exclusively with POC5A429. Protein disulfide-isomerase A4, Iduronate 2-sulfatase, Golgi resident protein GCP60, Aminopeptidase B and cDNA FLJ53442; highly similar to Poly (ADP-ribose) polymerase.
Charlotte Zaouter (182378), Hélène Mathieu (6433280), Edward T. Bagu (521821), Amani Hassan (5545277), Sirinart Molidperee (6433283), Isabelle Villemure (84134), Florina Moldovan (58849), Stefan Parent (4282717), Shunmoogum A. Patten (148312), Soraya Barchi (6433286)   +9 more
core   +1 more source

Dose-dependent effects of a brain-penetrating iduronate-2-sulfatase on neurobehavioral impairments in mucopolysaccharidosis II mice. [PDF]

Mol Ther Methods Clin Dev, 2022
Morimoto H, Morioka H, Imakiire A, Yamamoto R, Hirato T, Sonoda H, Minami K.   +6 more
europepmc   +1 more source