bluuebunny/crossref_metadata_2025_split huggingface dataset:
Electron transfer as a possible initial step in nucleophilic addition elimination reactions between (radical) anions and carbonyl compounds in the gas phase
Authors: Staneke Paul O. , Ingemann Steen , Nibbering Nico M. M. 
Published: September 2010

AbstractThe reactions of the HO−, CH3S−, CH2S− and CH2  C(CH3)CH2− ions with three ketones (CF3COR; R  CH3, CF3, C6H5) and three esters of trifluoroacetic acid (CF3CO2R; R  CH3, C2H5 and C6H5) have been studied with use of Fourier Transform Ion Cyclotron Resonance (FT‐ICR) mass spectrometry. All four negative ions react exclusively by proton transfer with CF3COCH3. With the other substrates, the HO− ion reacts by various pathways, such as proton transfer, SN2 substitution, E2 elimination and attack on the carbonyl group. The CH3S− ion is unreactive towards CF3COC6H5 but is able to react by hydride transfer, SN2, E2 and/or carbonyl attack with the remaining neutral species. The CH2S− radical anion reacts by electron transfer to afford stable molecular radical anions of CF3COCF3 and CF3COC6H5, whereas the main reaction with the two esters, CF3CO2CH3 and CF3CO2C2H5, is dissociative electron transfer leading to CF3CO2− and CF3− ions. The CH2C(CH3)CH2− anion displays a more complex reactivity pattern involving electron transfer, SN2, E2 as well as attack on the carbonyl group. Direct evidence for the occurrence of electron transfer as the initial step in an overall BAC2 type process has not been obtained for the systems studied. The reaction of the CH2S− ion with CF3CO2C6H5 was observed, however, to yield exclusively a CF3COCHS−. radical anion. Based upon the absence of a BAC2 process in the reaction of CH2S− with the methyl and ethyl esters of trifluoroacetic acid in combination with the facile occurrence of electron transfer from this radical anion, it is suggested that the CF3COCHS−. ion is formed by an initial electron transfer followed by coupling between the CH2S molecule and the CF3CO2C6H5− radical anion and subsequent loss of C6H5OH from the collision complex.
Ring expansion of norpenicillin to 3‐norcephalosporin
Authors: van der Klein Pieter A. M. , Castelijns Judith , Hirs Hans G. J. , Verweij Jan , de Vroom Erik 
Published: September 2010

AbstractThe Morin ring expansion of penicillin 1‐oxides to cephalosporins has been extended to norpenicillin 1‐oxides. To this end, both the norpenicillin sulfoxide moiety and the methyl group should be β‐oriented. Norpenicillin 1‐oxides are prepared from properly protected exomethylene penam derivatives using hydrogen on palladium/carbon and oxidation with 3‐chloroperoxybenzoic acid or peroxyacetic acid.
Metal‐binding affinity of a series of bis(2‐pyridylalkyl)amines immobilized on silica
Authors: Hoorn H.J. , de Joode P. , Driessen W.L. , Reedijk J. 
Published: September 2010

AbstractFour potentially tridentate bis(2‐pyridylalkyl)amines, differing in the number of methylene groups in the alkyl chain, have been coupled to the epoxy‐containing bifunctional spacer (3‐glycidoxypropyl)trimethoxysilane (GLYMO) and subsequently grafted onto silica. These ion exchangers were characterized by solid‐state CP‐MAS 13C‐NMR and elemental analyses. The obtained ligand concentrations varied from 0.29‐0.63 mmol/g ion exchanger. With the only exception of Alfa‐GLYMO‐bis(2‐pyridyl)amine, which showed a very low capacity for any of the metal ions tested, these ion‐exchange materials selectively absorbed Cu2+, with a maximum capacity of 0.22‐0.59 mmol/g ion exchanger at pH 5.6, from aqueous solutions containing a mixture of the divalent metal ions Cu2+, Ni2+, Co2+, Cd2+ and Zn2+. The metal‐uptake capacities and distribution coefficients established at different pH, correlate with the length of the alkyl bridge in the chelating ligand. The longer the alkyl spacer the lower the capacity and the metal‐ligand complex stability at lower pH. The use of strong chelating tridentate N‐donor ligands greatly increases the ligand occupation by metal ions. The Cu2+ uptake varies strongly with pH in the case of Alfa‐GLYMO‐bis[2‐(2‐pyridyl) ethyl]amine.
The oxidation of penicillin‐G‐potassium salt using supported polyoxometalates with hydrogen peroxide
Authors: Brown Scott W. , Johnstone Alexander , Jones Craig W. , Lee Angela M. , Oakes Steven C. , Wilson Sharon L. 
Published: September 2010

AbstractThe oxidation of the sulfide group in penicillin G potassium salt to the sulfoxide with hydrogen peroxide has been investigated using a wide range of polyoxometalates immobilized on high‐surface‐area supports. The highest activity and selectivity for the sulfur oxidation of the penicillin compound was obtained with ammonium molybdate supported on to strongly basic anion‐exchange resin. The oxidation proceeds smoothly at ambient and sub‐ambient temperatures in water or water/alcohol solvents. The use of supported oxidation catalysts has been extended to the transformation of benzenethiol to benzenesulfonic acid which was found to proceed in high yield and selectivity employing phosphotungstic acid on γ‐alumina. The use of a supported catalyst with H2O2 as the primary oxidant allows the circumvention of environmental and product isolation problems to be overcome simply and economically.
Gas‐phase hydrogenolysis mediated by activated carbon: Deuterated benzenes
Authors: Arends Isabel W. C. E. , Louw Robert , Mulder Peter 
Published: September 2010

AbstractHydrogenolyses of hexadeuterobenzene C6D6 and pentadeuterochlorobenzene C6D5Cl have been studied in flow reactors packed with activated carbon (AC) between 300° and 600°C, with void residence times of 4‐5 s. At 579°C overall dedeuteration ‐ proceeding in a near statistical manner ‐ was close to 40% in both cases, whereas C6D5Cl is almost completely dechlorinated to deuterated benzenes at 505°C. On a per‐site basis, replacement of Cl by H is ca. 20 times faster than that of D.These results clearly demonstrate a catalytic activity of AC in both cases of desubstitution. In the absence of AC, temperatures of 700‐900°C are necessary to obtain the same result. Then, replacement of Cl and D have almost equal rates, both reactions occurring via addition of free H atoms. In the AC‐mediated reactions, the yields of (D) benzenes are quantitative from C6D6, but around 50% with C6D5Cl. Apparently the latter compound reacts with bonding of its phenyl group to the AC surface, whereas D → H substitutions involve H transfer to a physisorbed (D) benzene.
Synthesis and characterization of 9,10‐bis(arylimino)‐9,10‐dihydrophenanthrenes, the structure of (Z,Z)‐9,10‐bis(phenylimino)‐9,10‐dihydrophenanthrene and PdCl2‐[(E,E)‐9,10‐bis(phenylimino)‐9,10‐dihydrophenanthrene] in the solid state and in solution
Authors: van Belzen Ruud , Klein René A. , Smeets Wilberth J. J. , Spek Anthony L. , Benedix Roland , Elsevier Cornells J. 
Published: September 2010

AbstractThe synthesis and characterization of substituted phenanthrene‐9,10‐quinone diimines of the type 9,10‐bis(arylimino)‐9,10‐dihydrophenanthrene (aryl‐BIP) is described. These rigid bisnitrogen ligands have been synthesized by a metal‐mediated cyclodehydrogenation reaction from the aromatic open‐chain diimine analogues derived from benzil. All aryl‐BIP compounds adopt a Z,Z configuration in solution except for (o‐iPr‐phenyl)‐BIP, which mainly exists in the E,Z form. All non‐cyclic BIP compounds undergo a configurational change into the E,E isomer upon coordination to palladium. NDDO calculations on phenyl‐BIP and its palladium dichloride complex have revealed that the energy of the LUMO is very much dependent on the puckering of the phenanthrene backbone and is in all cases lower than, for instance, that of the aromatic diimine of acenaphthene‐1,2‐quinone or open‐chain diimines. The structures of phenyl‐BIP and PdCl2‐(phenyl‐BIP) in the solid state have been determined by X‐ray diffraction. Phenyl‐BIP is triclinic, space group P̄, a = 12.392(1), b = 12.478(1), c = 14.633(2) Å, α = 109.36(1), β = 91.57(1), γ = 115.82(1)°, Z = 4, final R = 0.0556 for 4494 reflections with I > 2.5σ(I). PdCl2‐(phenyl‐BIP) is monoclinic, space group P21/c, a = 20.5148(9), b = 10.6756(9), c = 23.2007(16) Å, β = 105.13(1)°, Z = 4, final R = 0.0701 for 2569 reflections with I > 2.5σ(I).
Spontaneous crystallization in undercooled liquid mixtures of l‐limonene + l‐carvone
Authors: Calvet T. , Cuevas‐Diarte M.A. , Gallis H.E. , Oonk H.A.J. 
Published: September 2010

AbstractThe two related monoterpenes l‐carvone (m.p. −29°C) and l‐limonene (m.p. −74°C) when cooled to −120°C in DSC experiments remain in the liquid state. On subsequent heating to room temperature carvone crystallizes spontaneously at −75°C whereafter it melts at −29°C. Limonene, on the other hand, does not crystallize at all; to understand its behaviour, mixtures with carvone were investigated.
Facile synthesis of 2‐substituted benzoxazoles via ketenes
Authors: Olagbemiro T.O. , Agho M.O. , Abayeh O.J. , Amupitan J.O. 
Published: September 2010

AbstractThe generation of diphenyl‐, phenyl‐, phenoxy‐, and chloroketenes by treatment of the corresponding acid chlorides with triethylamine in the presence of 2‐aminophenol resulted in good yields of 2‐substituted benzoxazoles.
Synthesis of niruriside a HIV REV/RRE binding inhibitor
Authors: Duynstee H.I. , Ovaa H. , van der Marel G.A. , van Boom J.H. 
Published: September 2010

AbstractRegioselective acylation of 2,1′:4,6‐di‐O‐isopropylidene‐D‐sucrose (2) with trans‐cinnamoyl chloride gave intermediate 3, which was transformed in four steps into niruriside (1) in 23% overall yield. On the other hand, conversion of 2 into 1′,2,4,6‐tetra‐O‐acetylsucrose 10 followed by cinnamoylation gave (1) in 41% overall yield.
Separation of 2‐ and 4‐hydroxymandelic acid
Authors: Hoefnagel A.J. , Peters J.A. , van Bekkum H. 
Published: September 2010

AbstractHydroxyalkylation of phenol with glyoxylic acid, catalyzed by AlIII, results, under neutral aqueous conditions, in ortho‐rich mixtures of hydroxymandelic acids. Para‐rich mixtures of hydroxymandelic acids are obtained by ZnII catalysis under slightly alkaline conditions. A new separation method of these mixtures into the pure alkali salts of 2‐ and 4‐hydroxymandelic acid is presented. Formation of a complex between the alkali ion and three co‐operating oxygen atoms of 2‐hydroxymandelate explains the excellent solubility of the ortho isomer in acetone, enabling extraction of this isomer selectively from mixtures of alkali salts of 2‐ and 4‐hydroxymandelic acid.
Receptors functionalized with chiral aza‐crown ether rings. Attempted enantioselective catalysis of a Michael addition reaction
Authors: Schuurman Ruud J. W. , Grimbergen Reinier F. P. , Scheeren Hans W. , Nolte Roeland J. M. 
Published: September 2010

AbstractA series of receptors functionalized with chiral aza‐crown ether rings was synthesized. These compounds were studied as enantiopure catalysts for the addition of benzenethiols to cyclohex‐2‐en‐1‐one. Binding of 4‐hydroxybenzenethiol in these molecules allows for the orientation of the thiol with respect to an asymmetric catalytic site. This orientation was, however, found to be counterproductive for enantioselective catalysis.
1‐Alkyl‐4‐(alkylamino) quinolinium‐3‐thiolates
Authors: Maślankiewicz Andrzej , Ziȩba Andrzej , Glowiak Tadeusz 
Published: September 2010

AbstractReaction of 5,12‐dialkylthioquinanthrenediinium bis‐salts 1 with primary alkylamines led to the synthesis of 1‐alkyl‐4‐(alkylamino)quinolinium‐3‐thiolates (2). Their structures were proved by X‐ray examination, 1H‐NMR spectroscopy and by transformation to 1‐alkyl‐4‐(alkylamino)‐3‐(alkylthio)quinolinium salts (3).
Oxidation catalysis by biomimetic manganese complexes
Authors: Hage Ronald 
Published: September 2010

AbstractA review is given of certain aspects of manganese‐containing enzymes, such as manganese superoxide dismutase, manganese catalase, and the photosynthetic oxygen‐evolving catalyst. A variety of manganese complexes have been synthesized to model either the structure of the active site of these enzymes (structural models), or their function (functional models). Most of the enzymatic reactions can be mimicked by functional models, albeit at much lower reaction rates. Additionally, a number of manganese complexes based on the structural mimics, have been studied as catalysts in oxidation reactions other than the enzymatic ones.Examples of functional models for superoxide dismutase, catalase and the water oxidation catalyst in plants, as well as a number of manganese complexes catalyzing a variety of other oxidation reactions, are also discussed in this review.
Solid‐support synthesis of di‐ and tetramannosylated tetrathymidylic acid
Authors: Wijsman E.R. , Filippov D. , Valentijn A.R.P.M. , van der Marel G.A. , van Boom J.H. 
Published: September 2010

AbstractThe easy accessible building units 5‐O‐(2,3,4,6‐tetra‐O‐benzoyl‐α‐D‐mannopyranosyl)penyl 2‐cyanoethyl N,N‐diisopropylphosphoramidite (9) and 2,2‐bis(4,4′‐dimethoxytrityloxymethyl)propyl 2‐cyanoethyl N,N‐diisopropylphosphoramidite (12) were used for the sequential elongation of immobilized tetrametric thymidylic acid according to a DNA synthesis protocol. Ammonolysis of the individual immobilized adducts led to the isolation of the target compounds 1 and 2.
5,17‐Bis(acetamido)‐25,26,27,28‐tetrapropoxycalix[4]arene; the first example of two different pinched cone conformations in the solid state
Authors: Verboom Willem , Vreekamp Remko H. , Bodewes Paul J. , Reinhoudt David N. , Harkema Sybolt 
Published: September 2010

AbstractCrystals of 5,17‐bis(acetamido)‐25,26,27,28‐tetrapropoxycalix[4]arene · ethyl‐acetate (1: 1), C44H54N2O6 · C4H8O2 are triclinic, space group P‐1, a = 13.319(4), b = 15.519(4), c = 22.386(6) Å, α = 104.73(3), β = 99.02(3), γ = 95.30(3)°, T = 100(5) K, Z = 4. The asymmetric unit contains two calix[4]arene molecules and two ethyl acetate molecules. The two calix[4]arene molecules have different pinched cone conformations. The phenyl rings show small but consistent boat‐like deformations. The final R factor is 7.9%.
Chemo‐ and diastereoselective cyclic ketalization of optically pure 1‐aryl‐2,2‐dimethylpropane‐1,3‐diols with phenylglyoxal
Authors: Xianming Hu , Kellogg Richard M. 
Published: September 2010

AbstractReactions of optically pure 1‐aryl‐2,2‐dimethylpropane‐1,3‐diols with phenylglyoxal give diastereomerically pure cyclic ketals under acid catalyzed conditions. The structure of one of the products, 2‐phenyl‐4‐(2‐chlorophenyl)‐5,5‐dimethyl‐1,3‐dioxane‐2‐carbaldehyde (7), has been determined by X‐ray crystallography; reaction takes place at the keto group and the aldehyde group is oriented axially.
Selective de‐oxygenation of organic compounds
Authors: Ponec V. 
Published: September 2010

AbstractIn this paper, recent results on the de‐oxygenation of some organic molecules nitrobenzene, aliphatic carboxylic acids and benzoic acid are reviewed. The activity pattern of metal oxides in these reactions has been established, and several factors controlling the selectivity have been identified. Selectivity in the de‐oxygenation (and related) reaction is related to the universally operating Mars ‐ Van Krevelen mechanism.
Promoted platinum catalysts for automotive pollution control. Metal oxides of Co, Cr, Al, Mn, Mg, Ce, Zr, V, La, Ti and Fe as additives
Authors: Mergler Y.J. , Chandoesing R. , Nieuwenhuys B.E. 
Published: September 2010

AbstractExploratory research to develop Rh‐free, “Pt‐only”, automotive catalysts is described. Metal oxides were added as a promoter or co‐catalyst to Pt/SiP2 and the effect of the additive on NO reduction by CO has been studied. In addition, the reaction was studied over the unsupported metal oxides (MO) and Pt/MO catalysts. From the series of metal oxides tested, it is concluded that Co, Fe, Mn, Ti and V oxides show promising results as additives to Pt for the catalysis of NO reduction by CO.
Sorption of alkanes on novel pillared zeolites; comparison between MCM‐22 and MCM‐36
Authors: Eder Florian , He Yingjie , Nivarthy Gautam , Lercher Johannes A. 
Published: September 2010

AbstractMCM‐22 was synthesized and modified by swelling and pillaring to obtain MCM‐36. These two structures were characterized by means of XRD, SEM, XRF, nitrogen adsorption, NH3 adsorption/desorption and IR spectroscopy. The sorption of light alkanes on the protonic form of MCM‐22 and MCM‐36 was investigated using gravimetry, calorimetry and in situ IR spectroscopy. Distinct differences in the properties of the two materials are observed. Swelling and pillaring of MCM‐22 leads to an increase in crystal size and mesopore volume, while the micropore volume and the concentration of the Brønsted acid sites decreases. The acid sites are found to be sited to a large extent in the 10‐membered‐ring channels of MCM‐22. This channel system is present in MCM‐22 and MCM‐36. As alkanes are sorbed in the same local environment in both samples, identical enthalpies of adsorption are observed. The enthalpy of adsorption varies with the entropy of sorption in a manner typical for structures consisting of 10‐membered‐ring channels. This indicates that the localized part of the alkane sorption properties on MCM‐22 and MCM‐36 is dominated by the 10‐membered‐ring channel system.
Über Komplexverbindungen im System Cr3+/Cl‐ in Dimethylsulfoxyd
Authors: Schläfer H. L. , Opitz H. P. 
Published: September 2010

AbstractEs wird gezeigt, daß CrCl3 in wasserfreiem Dimethylsulfoxyd (DMSO) bei Zimmertemperatur praktisch als cis‐[Cr(DMSO)4Cl2]+‐ Ion vorliegt. Bei der aus den Lösungen auskristallisierenden grauvioletten Verbindung CrCl3 · 5 DMSO handelt es sich, wie aus dem Vergleich des Lösungs‐ mit dem Reflexionsspektrum folgt, um cis‐[Cr(DMSO)4Cl2]Cl · DMSO. Durch thermische Behandlung dieser Verbindung entsteht rotviolettes CrCl3 · 3 DMSO, das als Nichtelektrolytkomplex [Cr(DMSO)3Cl3] zu formulieren ist.In den Lösungen von cis‐[Cr(DMSO)4Cl2]+ existiert das reversible Gleichgewicht cis‐[Cr(DMSO)4Cl2]+ + Cl‐ ⇌ [Cr(DMSO)3Cl3] + DMSO, das sich bei höheren Temperaturen zum rotvioletten Trichlorokomplex verschiebt. Das Gleichgewicht wird mit Hilfe von spektrophotometrischen und von Leitfähigkeitsmessungen untersucht.
Physikalische Stabilität und Elektronenverteilung in homonuklearen mehratomigen linearen Molekülen
Authors: Müller Hans 
Published: September 2010

AbstractMit Hilfe eines einfachen dreidimensionalen FE‐Modells werden die im Kohlenstoffdampf auftretenden linearen Moleküle Ck (k = 1, 2, 3, …) behandelt. Im Anschluß an die Untersuchung der physikalischen Stabilität findet eine ausführliche Diskussion der Elektronenverteilung in diesen Molekülen statt, die qualitativ an Hand des Bindungscharakters der FE‐MO's und quantitativ mittels einer Elektronenpopulationsanalyse speziell für C3 und C4 durchgeführt wird. Die Ergebnisse entsprechen der Struktur : C = C = … = C:. Ferner findet ein Vergleich mit den linearen Aktivierungskomplexen H3 und H4 statt.
Über die Möglichkeiten der röntgenspektroskopischen Untersuchung von Katalysatoren
Authors: Gáti Gy. , Meisel A. 
Published: September 2010

AbstractIm ersten Teil dieser Arbeit werden Überlegungen angestellt, in wieweit die Methoden der Röntgenemissions‐ und ‐absorptionsspektroskopie zur Aufklärung der elektronischen Struktur von Katalysatoren und ihrer Änderung durch Wechselwirkungen des Katalysators mit Trägersubstanzen und chemisorbierten Molekülen beitragen können. Im zweiten Teil werden weitere Möglichkeiten der röntgenspektroskopischen Katalysatoruntersuchung am Beispiel des WS2 beschrieben. Durch Aufnahme des S Kα‐Dubletts wird die Möglichkeit der Bestimmung der Konzentration verschiedener Wertigkeitsstufen erörtert, und auf Grund der W L‐Emissions‐ und ‐Absorptions‐spektren wird die elektronische Struktur des Wolframdisulfids im Zusammenhang mit seinen katalytischen Eigenschaften diskutiert.
Flüssige Ionenaustauscher — I. Wesen und Anwendung
Authors: Häupke Klaus , Wolf Friedrich 
Published: September 2010

AbstractDie Bezeichnung „flüssige Ionenaustauscher”︁ hat sich in letzter Zeit für eine Gruppe ionenselektiver Extraktionsmittel eingeführt, die aus Lösungen von nicht wasserlöslichen ionogenen Verbindungen (langkettigen sauren Phosphorsäureestern oder langkettigen Aminen) in mit Wasser nicht mischbaren Lösungsmitteln be‐stehen. Sie eignen sich speziell zur Extraktion von Metallen zum Zweck der Gewinnung, Reinigung oder Trennung auf nassem Wege. Es wird ein Überblick über die bekanntgewordenen flüssigen Ionenaustauscher, ihre Anwendung und ihre Wirkungsweise gegeben.
Interhalogenverbindungen
Authors: Meinert Hasso 
Published: September 2010

AbstractVon den binären Interhalogenen sind die Typen AB, AB3, AB5und AB7, von den ternären die Verbindungen JCIF und JCIF2bekannt.Über Interhalogene sind bereits mehrere Übersichts‐ bzw. Teilübersichtsartikel erschienen [1]‐[10]. In den letzten Jahren ist aber so viel Neues über diese Verbindungsklasse berichtet worden, daß eine Zusammenfassung angebracht erscheint.
Fraktionierung, Struktur und mögliche biologische Funktion der Histone
Authors: Schwenke Klaus Dieter 
Published: September 2010

AbstractForschungsstelle für Experimentelle Onkologie der Deutschen Akademie der Wissenschaften zu Berlin in Potsdam‐Rehbruckejetzt: Arbeitsgruppe Dr. med. habil F. Schmidt im Institut für Ernährung der DAW zu Berlin in Potsdam‐Rehbrücke.
Vergleichende Untersuchungen zur Einsatzmöglichkeit des kaskadenstabilisierten Lichtbogens
Authors: Doerffel Klaus , Gehlsen‐Lorenzen Hans‐Peter Timm , Mann Ute 
Published: September 2010

AbstractIm Verlaufe bisheriger Untersuchungen hat sich der kaskadenstabilisierte Lichtbogen nach Riemann [1] als wertvolles analytisches Hilfsmittel erwiesen. Im folgenden soll versucht werden, die Eigenschaften dieser Lichtquelle in den Vergleich mit Verbrennungsflamme, Plasmastrahlerzeuger und Atomabsorption zu stellen und hieraus Rückschlüsse auf jeweils optimale Einsatzmöglichkeit abzuleiten.
Long‐term observational, non‐randomized study of enzyme replacement therapy in late‐onset glycogenosis type II
Authors: Bembi Bruno , Pisa Federica Edith , Confalonieri Marco , Ciana Giovanni , Fiumara Agata , Parini Rossella , Rigoldi Miriam , Moglia Arrigo , Costa Alfredo , Carlucci Annalisa , Danesino Cesare , Pittis Maria Gabriela , Dardis Andrea , Ravaglia Sabrina 
Published: September 2010

AbstractObjectivesType II glycogenosis (GSDII) is a lysosomal storage disorder due to acid alpha‐glucosidase (GAA) deficiency. Enzyme replacement therapy (ERT) with human recombinant alpha‐glucosidase (rhGAA) has been demonstrated to be effective in the treatment of infantile forms of GSDII, but little information is available concerning late‐onset phenotypes. Long‐term follow‐up studies are not available at present. The aim of this study was to evaluate the ERT long‐term effects in late‐onset GSDII.MethodsTwenty‐four patients, including 7 juveniles and 17 adults, received bi‐weekly infusion of rhGAA (20 mg/kg) for at least 36 months. Clinical conditions, muscular function (6‐min walking test, 6MWT; Walton scale, WS), respiratory function (vital capacity, VC; forced expiratory volume, FEV1; arterial pCO2), and muscle enzymes were assessed every 6 months.ResultsThe 6MWT improved in both juvenile and adult patients (p = 0.01, p = 0.0002, respectively), as well as in patients with moderate to severe muscle function impairment (WS > 3.5; p = 0.002). An overall improvement in WS was also observed (p = 0.0003). VC and FEV1 remained unchanged, while pCO2 decreased (p = 0.017). Muscle enzymes decreased significantly (p < 0.0001). Two patients (8%) showed transient secondary events during ERT.ConclusionsLong‐term ERT with rhGAA was shown to be safe, well tolerated, and effective in improving motor function and in stabilizing respiratory function in late‐onset GSDII. The response pattern showed a progressive clinical improvement during the follow‐up period in juvenile patients, while in adults it reached and maintained a plateau after the first year of treatment.