ライフサイエンスコーパス: MPS

1 MPS IH is characterized by a broad spectrum of clinical

2 MPS IVA fibroblasts treated with these chaperones exhibi

3 MPS models representing the major absorption, metabolism

4 MPS peptide was demonstrated to impair cell proliferatio

5 MPS type I patients are prone to have glaucoma with narr

6 MPS types I, IV, and VI had higher IOP estimates than th

7 MPS typically segregates as an autosomal-recessive disor

8 MPS-I and -II groups were further subdivided according t

9 d O ( approximately 1.35 +/- 0.1% day(-1) ), MPS increased in response to RET only in Y (3 weeks, Y:

10 atient samples (MPS-I n = 18, MPS-II n = 12, MPS-VI n = 6, control n = 20).

11 ion cohort of patient samples (MPS-I n = 18, MPS-II n = 12, MPS-VI n = 6, control n = 20).

12 Such 1D MPS was also observed in dendrites, but the extent to wh

13 We observed that the 1D MPS exists in a substantial fraction of dendritic region

14 and dendrites than the development of the 1D MPS in axons.

15 This 1D MPS structure was initially observed in axons and exists

16 simple multiparametric scoring function (AB-MPS) was devised that correlated preclinical PK results

17 Abnormal MPS perfusion was defined as a summed stress score >=4,

18 erall, 14% (n=2177) of patients had abnormal MPS of whom 11% (n=1698) had ischemia and 4% (n=684) ext

19 was not an independent predictor of abnormal MPS.

20 For patients with abnormal MPS results, at a 50% reduction there was a significant

21 ggregation and separation protocols activate MPS cells.

22 Even by 1 month of age, MPS IIIB dogs had elevated HS levels in brain and cerebr

23 allow newborn screening and diagnosis of all MPS disorders except the ultrarare MPS-IX.

24 Assay of all MPS enzymes using fluorimetric or other methods has not

25 Exercise (0.012 +/- 0.013%/h) (P < 0.01) and MPS was greater in WP than CP in both the Rest and Exerc

26 cally significant difference between CMR and MPS strategies.

27 Linear, as opposed to branched, MOS and MPS were preferentially extracted under all conditions t

28 lues were significantly lower in the PKU and MPS groups than in controls and neurological patients.

29 ts with different IEM (especially in PKU and MPS patients, but also in IEM under protein-restricted d

30 4, which, over time, decreased basal RNA and MPS in a dose-dependent manner (correlation of RNA and M

31 ose-dependent manner (correlation of RNA and MPS, r(2) = 0.98), even though it had no effect on the a

32 have glaucoma with narrow or closed angles; MPS type II patients tend to have plateau iris; MPS type

33 ons causes a rapid disassembly of the axonal MPS, which occurs prior to protein loss and independentl

34 = 85); basiliximab (BAS)/EVR (N = 102); BAS/MPS (N = 101).

35 inical or subclinical WHAE (r-ATG/EVR vs BAS/MPS hazard ratio 1.30; BAS/EVR vs BAS/MPS hazard ratio 1

36 vs BAS/MPS hazard ratio 1.30; BAS/EVR vs BAS/MPS hazard ratio 1.73, P = 0.028).

37 3% vs 7% vs 4%, P = 0.025) compared with BAS/MPS, respectively.

38 vs 42% vs 26%, P = 0.014) compared with BAS/MPS, respectively.

39 CS group) or HMP (WI-HMP group) using Belzer-MPS solution.

40 FJPS), fruit juice (FJPS) and milk beverage (MPS), were stored at 4, 24, or 37 degrees C and analysed

41 markers significantly changed in human brain MPS after tolcapone dosing, which were mainly associated

42 In parallel, metabolomics in brain MPS evaluated the tolcapone influences on endogenous pat

43 m principal strain (MPS) of the whole brain, MPS of the corpus callosum, and fiber strain of the corp

44 nd the pathophysiology of glaucoma caused by MPS.

45 the abnormal neurite overgrowth exhibited by MPS neurons.

46 approximately 24-30 months of age) of canine MPS IIIB disease.

47 breeding practices, the prevalence of canine MPS IIIB has been drastically reduced in the Schipperke

48 Pharmacological studies using the cardiac MPS show half maximal inhibitory/effective concentration

49 ontrast, two of the mutations found to cause MPS in this study occurred in the tail domain.

50 Given that the best characterized MPS-I murine model is an immunocompetent mouse, we here

51 or the quantification precision of clinical MPS.

52 Under postabsorptive conditions, MPS and LPB were equivalent between groups, whereas insu

53 d enzymatic activities measured in confirmed MPS samples are well below the reference range.

54 This study enrolled 27 consecutive MPS patients (8 patients with MPS I, 4 patients with MPS

55 wed systematic biases with the corresponding MPS sequencing pipeline.

56 Moreover, D2O heralds promise for coupling MPS and muscle mass and providing insight into the contr

57 E), provide guidance for physically coupling MPS, and offer an approach to coupling MPS with distinct

58 pling MPS, and offer an approach to coupling MPS with distinct media and perfusion requirements.

59 Measurements of cumulative MPS, the sum synthesis over an extended period, using de

60 However, current MPS typically lack integrated sensors and their fabricat

61 K signaling in turn caused calpain-dependent MPS degradation, providing a negative feedback that modu

62 pared with a normal result (risk difference: MPS 20 per 100 patients tested [95% CI, 0.11-0.29], DSE

63 hoid tissues, CSF1R-FRed highlighted diverse MPS populations, including classical dendritic cells.

64 en identified, the genetic basis of dominant MPS remains unknown.

65 w ranges of MOS, lower DP MPS, and higher DP MPS were obtained through repetitive 70%-ethanol extract

66 th relatively narrow ranges of MOS, lower DP MPS, and higher DP MPS were obtained through repetitive

67 e mycophenolic acid absorption as well as EC-MPS.

68 orption of SRL and mycophenolic acid from EC-MPS were similar.

69 al symptoms with mycophenolate mofetil or EC-MPS in combination with Tac and cyclosporin, but this wa

70 ac), enteric-coated mycophenolate sodium (EC-MPS) and sirolimus (SRL) in oral dosage forms was well-p

71 ttenuation of MPB without robustly enhancing MPS or NPB.

72 root mean squared error (RMSE) of 0.014 for MPS of the whole brain.

73 group x time interactions) were detected for MPS or cell signaling.

74 this variant represent a promising model for MPS I with neurological abnormalities in humans.

75 elopment of next generation therapeutics for MPS.

76 This study explores a potential therapy for MPS-I at a very early stage in life and represents a nov

77 been useful to evaluate novel treatments for MPS IIIB, but has limitations.

78 Additional studies in cultured neurons from MPS I mice showed that elevated spermine was essential f

79 Electroretinogram (ERG) was recorded from MPS IIIB and wild-type (WT) mice at the age of 28 and 46

80 tification of HS in 5 muL urine samples from MPS patients and healthy controls.

81 ients are vulnerable to open-angle glaucoma; MPS type VI patients have narrow angles not as close as

82 ECT images were analyzed to calculate global MPS defect size and regional defect size for 3 coronary

83 d in Exercise (P < 0.0001) with WP; however, MPS was not significantly elevated above baseline in Res

84 In this study, five human MPS models were evaluated for functional coupling, defin

85 spects and limitations of implementing human MPS, especially in conjunction with in situ single cell

86 e studies demonstrate the potential of human MPS for multi-organ toxicity and absorption, distributio

87 Mucopolysaccharidosis type I (MPS-I) is a progressive multi-system disorder caused by

88 rler syndrome (mucopolysaccharidosis type I [MPS I]).

89 ndrome Type B or Mucopolysaccharidosis IIIB (MPS IIIB) is a neurodegenerative autosomal recessive lys

90 Mucopolysaccharidosis type IIIB (MPS IIIB; Sanfilippo syndrome B) is an autosomal recessi

91 In MPS I patients, elevated CSF spermine was restricted to

92 arkers for central nervous system disease in MPS patients would facilitate the evaluation of new agen

93 ghts into the pathogenesis of CNS disease in MPS patients, and support the use of spermine as a new b

94 The ordinary IOP estimates in MPS VI patients may be falsely high because of clouded c

95 es are reported, they can be incorporated in MPS models to maximize phenotype prediction.

96 F heparan sulfate and a 3-8 fold increase in MPS-IH specific non-reducing ends, I0S0 and I0S6.

97 phosphate pools and AKT activity occurred in MPS-treated IPF fibroblast cells.

98 rotein Heg1 was significantly raised only in MPS-VI.

99 suggest that inflammation may play a role in MPS pathophysiology.

100 f testing the therapeutic efficacy of UCB in MPS-I mice transplanted at birth, we first defined the f

101 ulin and amino acid administration increased MPS in only healthy-weight subjects and was associated w

102 rcise) (both P < 0.01), whereas CP increased MPS only in Exercise (0.012 +/- 0.013%/h) (P < 0.01) and

103 +/- 0.08%/d), the T leg exhibited increased MPS at 0-3 wk (1.6 +/- 0.01%/d), but not at 3-6 wk (1.29

104 Acutely, WP increased MPS by a mean +/- SD 0.017 +/- 0.008%/h in the feeding-o

105 type II patients tend to have plateau iris; MPS type IV patients are vulnerable to open-angle glauco

106 Mucopolysaccharidosis type IVA (MPS IVA) is a rare disease caused by mutations in the ge

107 ar Cardiology guidelines for (99m)Tc-labeled MPS is highly feasible while retaining short imaging pro

108 saccharides (MOS), and maltopolysaccharides (MPS).

109 Lean mass, MPS, LPB and strength were not different but work output

110 Ultra-low-dose (<190 MBq) MPS even with short imaging times (<6 min) is feasible u

111 yer of 3-methacryloxypropyltrimethoxysilane (MPS) and a second layer of tetraethylorthosilicate (TEOS

112 0.05) in all disease groups apart from mild MPS-I and -II.

113 een recently expanded to include one or more MPS disorders, as treatments are available and are most

114 ing (RNA-seq) data sets generated from mouse MPS cells isolated from bone marrow, blood, and multiple

115 was elevated in neuropathic subtypes of MPS (MPS I, II, IIIA, IIIB), but not in subtypes in which cog

116 The mucopolysaccharidoses (MPS) are lysosomal storage disorders that result from de

117 The mucopolysaccharidoses (MPS) are rare genetic disorders marked by severe somatic

118 (PKU), patients with mucopolysaccharidoses (MPS), patients with other inborn errors of metabolism (I

119 The mucopolysaccharidoses (MPSs) are a class of inborn errors of metabolism caused

120 Mucopolysaccharidosis (MPS) IIIB (Sanfilippo syndrome B; OMIM 252920), is a lys

121 Mucopolysaccharidosis (MPS) is a metabolic storage disorder caused by the defic

122 Mucopolysaccharidosis (MPS) type-IH is a lysosomal storage disease that results

123 of neutralizing mAbs to these CSFs on murine MPS populations in the steady-state and during acute inf

124 nsplantation in busulfan-conditioned newborn MPS-I mice.

125 is that transplanting normal BM into newborn MPS I mice soon after birth can prevent skeletal dysplas

126 For patients with normal MPS results, there were no differences in defect size, L

127 commented on the implications of this novel MPS classification in solid organ transplantation.

128 o a prolonged (6-36 h) elevation (30-50%) of MPS that was fully blocked by rapamycin at 6 h but only

129 A single variant was found in exon 6 of MPS IIIB affected Schipperkes: an insertion consisting o

130 The trabecular iris angles of MPS types I, II, and VI were smaller than those of MPS t

131 The angle recess areas of MPS types I and VI were smaller than those of MPS type I

132 Blunting of MPS in the obese men was offset by an apparent decline i

133 andial LGD in obese subjects and blunting of MPS responses to amino acids suggest that obesity in old

134 Urine samples from a small cohort of MPS-I, -II, and -VI patients (n = 12) were analyzed usin

135 Heparan sulfate from the cerebral cortex of MPS IIIA mice showed enhanced ability to increase glutam

136 The time course of MPS responses to exercise and the influence of training

137 large data sets to identify the diversity of MPS cellular phenotypes and the limited predictive value

138 erapy to improve the therapeutic efficacy of MPS IVA enzyme replacement therapy.

139 tive tissue are typical clinical features of MPS due to disruption of the extracellular matrix.

140 distinguish the severe neurological form of MPS-II from the milder non-neurological form.

141 , developmental requirements and function of MPS cells are beginning to solve this problem in an obje

142 ments for the neurological manifestations of MPS has been hindered by the lack of objective measures

143 l fluid (CSF) samples from a canine model of MPS I revealed a marked elevation of the polyamine, sper

144 et of behavioral changes in a mouse model of MPS IIIA.

145 ated the naturally occurring canine model of MPS IIIB for the onset and progression of biochemical an

146 e progression using the established model of MPS IIIB, the B6.129S6-Naglu(tm1Efn)/J mouse line.

147 urodevelopment, we examined murine models of MPS IIIA, which lack the enzyme sulfamidase.

148 activity was increased in visceral organs of MPS-I animals, glycosaminoglycans storage was reduced, a

149 est probability of CAD are not predictive of MPS perfusion abnormality and overestimate its prevalenc

150 We observed the presence of MPS in all of the tested neuronal types cultured from mo

151 Prevalence of MPS abnormality was substantially lower than expected ba

152 n profiling to probe for changes in rates of MPS and MPB at the individual protein level following ex

153 mine was elevated in neuropathic subtypes of MPS (MPS I, II, IIIA, IIIB), but not in subtypes in whic

154 life markedly reduces signs and symptoms of MPS I before they appear.

155 have narrow angles not as close as those of MPS type I.

156 pes I, II, and VI were smaller than those of MPS type IV and of the control subjects.

157 PS types I and VI were smaller than those of MPS type IV and of the control subjects.

158 The cone a- and b-waves of MPS IIIB mice were not significantly different from thos

159 We anticipate the widespread adoption of MPSs for drug screening and disease modeling.

160 The 6 patients in whom the placement of MPSs failed were retreated with a fully cover self-expan

161 resistance exercise and nutrition impact on MPS and MPB.

162 stimates than the control subjects, but only MPS I and IV had higher corrected IOP estimates than the

163 o guided care based on the results of CMR or MPS testing.

164 the MPS cluster, no more distinct than other MPS cells.

165 Our MPS is able to keep human induced pluripotent stem cell

166 rically, children with the severe phenotype, MPS-IH (Hurler syndrome) develop progressive neurodegene

167 ted with the highest prevalence for positive MPS (33% in men and 14% in women), ischemia (30% in men

168 Bed rest decreased postabsorptive MPS by 30% +/- 9% (CON group) and by 10% +/- 10% (LEU gr

169 es in which cognitive function is preserved (MPS IVA, VI).

170 r degeneration; actin stabilization prevents MPS disassembly and retrograde signaling during TD.

171 e taken at 3 and 6 wk to temporally quantify MPS via gas chromatography:pyrolysis:isotope ratio mass

172 Whereas several mutations causing recessive MPS have been identified, the genetic basis of dominant

173 ts such as omega-3 fatty acids in regulating MPS.

174 ntaining up to 37.5% of encapsulated roasted MPS pea starch not only provided high SDS and RS fractio

175 larger validation cohort of patient samples (MPS-I n = 18, MPS-II n = 12, MPS-VI n = 6, control n = 2

176 abnormal myocardial perfusion scintigraphy (MPS), dobutamine stress echocardiography (DSE) or corona

177 re, but here we use a multi-polygenic score (MPS) approach to increase predictive power by exploiting

178 MARCKS phosphorylation site domain sequence (MPS), to determine if MARCKS inhibition reduces pulmonar

179 Accurate massively parallel sequencing (MPS) of genetic variants is key to many areas of science

180 Massively parallel sequencing (MPS) technologies including custom, targeted gene panels

181 ein 7 concentrations were elevated in severe MPS I and II groups.

182 ite its strong presence in the axonal shaft, MPS is disrupted in most presynaptic boutons but is pres

183 Neurons have a membrane periodic skeleton (MPS) composed of actin rings interconnected by spectrin.

184 orm a membrane-associated periodic skeleton (MPS) in neurons.

185 orm a membrane-associated periodic skeleton (MPS) in neurons.

186 based Membrane-associated Periodic Skeleton (MPS), and effects of actin and spectrin manipulations in

187 this membrane-associated periodic skeleton (MPS), it is important to address how prevalent this stru

188 ng everolimus (EVR) or mycophenolate sodium (MPS).

189 on exposure from myocardial perfusion SPECT (MPS).

190 Using the modulation power spectrum (MPS [4, 5]), a recently developed, neurally informed cha

191 y at a relatively late state in steady-state MPS development; in contrast, GM-CSF neutralization had

192 s were treated with multiple plastic stents (MPSs) with a success rate of 62.5% (10 patients).

193 sidered, including maximum principal strain (MPS) of the whole brain, MPS of the corpus callosum, and

194 tients without known CAD referred for stress MPS for suspected CAD between 2004 and 2011 were identif

195 er, survivors remain burdened by substantial MPS-IH related residual disease.

196 ucopolysaccharidosis type I-Hurler syndrome (MPS-IH) is a lysosomal storage disease characterized by

197 ucopolysaccharidosis type I-Hurler syndrome (MPS-IH), along with an analysis defining a path to bette

198 HCT) benefits children with Hurler syndrome (MPS-IH).

199 Multiple pterygium syndrome (MPS) is a phenotypically and genetically heterogeneous g

200 Sanfilippo syndrome, MPS IIIA-D, results from deficits in lysosomal enzymes t

201 me biogenesis, and muscle protein synthesis (MPS) and degradation.

202 dy that determined muscle protein synthesis (MPS) and leg protein breakdown (LPB) under postabsorptiv

203 osal, muscle myofibrillar protein synthesis (MPS) and leg protein breakdown (LPB) were measured durin

204 dinated changes in muscle protein synthesis (MPS) and muscle protein breakdown (MPB).

205 Muscle protein synthesis (MPS) fluctuates widely over the course of a day and is i

206 sponse of skeletal muscle protein synthesis (MPS) to anabolic stimuli such as protein ingestion (and

207 weeks to quantify muscle protein synthesis (MPS) via gas chromatography-pyrolysis-isotope ratio mass

208 Muscle protein synthesis (MPS) was measured using [1, 2-(13) C2 ] leucine with bre

209 ation and skeletal muscle protein synthesis (MPS), can protect skeletal muscle health during bed rest

210 developed a human microphysiological system (MPS) model of the human liver acinus, a common metastati

211 present a cardiac microphysiological system (MPS) with the attributes required for an ideal in vitro

212 modulating the mononuclear phagocyte system (MPS) accumulation of polyion complexes.

213 The mononuclear phagocyte system (MPS) comprises monocytes, macrophages and dendritic cell

214 uptake by the mononuclear phagocyte system (MPS) in vivo with a long blood circulation half-life of

215 The mononuclear phagocyte system (MPS) is a family of cells including progenitors, circula

216 The mononuclear phagocyte system (MPS) is defined as a cell lineage in which committed mar

217 posited in the mononuclear phagocyte system (MPS) such as the liver and spleen.

218 unction of the mononuclear phagocyte system (MPS).

219 The mononuclear phagocyte system (MPS, e.g., liver, spleen) is often treated as a "blackbo

220 f cells of the mononuclear phagocyte system (MPS; progenitors, monocytes, macrophages, and classical

221 Human microphysiological systems (MPS) can model these interactions and are predicted to d

222 Recently, microphysiological systems (MPS), also known as organs-on-chips, that recapitulate t

223 even interacting microphysiological systems (MPSs), brain, pancreas, liver, lung, heart, gut, and end

224 While 'basal' longer term MPS was identical between Y and O ( approximately 1.35 +

225 E-induced increases of acute and longer-term MPS after ingestion of whey protein (WP) and collagen pr

226 Longer-term MPS increased by 0.063 +/- 0.059%/d in Rest and 0.173 +/

227 Longer-term MPS was greater in WP than in CP in both Rest and Exerci

228 early stages of RET, reflecting longer-term MPS.

229 who had undergone stress (99m)Tc-tetrofosmin MPS were postprocessed.

230 Surprisingly, we found that MPS peptide decreases alpha-SMA expression and synergist

231 he same lung cell populations indicated that MPS heterogeneity implied by global cluster analysis may

232 A sample-to-sample matrix revealed that MPS populations could be separated based upon tissue of

233 We also show that MPS destabilization through NMII inactivation affects ax

234 Quantitative analyses show that MPS is preferentially formed in axons in all neuronal ty

235 The MPS approach predicted 10.9% variance in educational ach

236 The MPS approach should be useful in research with modest sa

237 resulting in the preferential loss among the MPS populations of a cycling, monocyte-derived inflammat

238 njected nanomaterials are sequestered by the MPS, preventing their delivery to the desired disease ar

239 95% CI, 6.4%-11.6%; 42/481 patients) for the MPS group.

240 (17.7% [95% CI, 14.4%-21.4%]); and 78 in the MPS group (16.2% [95% CI, 13.0%-19.8%]).

241 roup, 2.5% in the CMR group, and 2.5% in the MPS group (adjusted hazard ratios: CMR group vs NICE gui

242 7.5%) in the CMR group, and 34 (7.1%) in the MPS group; adjusted odds ratio of unnecessary angiograph

243 neovascularization were also detected in the MPS IIIB retina.

244 In the MPS, short actin filaments, capped by actin-capping prot

245 markers and transcriptomic diversity in the MPS, we collected from National Center for Biotechnology

246 we show that in rat hippocampal neurons the MPS is an actomyosin network that controls axonal expans

247 s reveal an important functional role of the MPS and establish it as a dynamically regulated platform

248 ata demonstrate structural plasticity of the MPS and suggest its potential role in early steps of axo

249 decades, classification of the cells of the MPS has generated considerable controversy.

250 Cells of the MPS lineage have been identified using numerous surface

251 studies have questioned the validity of the MPS model and argued that tissue-resident macrophages ar

252 Disruption of the MPS prevented such molecular colocalizations and downstr

253 The function of the MPS, however, remains poorly understood.

254 n of betaII-spectrin, a key component of the MPS, suppresses retrograde signaling and protects axons

255 with exquisite specificity for cells of the MPS.

256 We conclude that the MPS remains a valid and accurate framework for understan

257 ed signaling molecules were recruited to the MPS in response to extracellular stimuli, resulting in c

258 urprisingly, attenuation of MARCKS using the MPS (MARCKS phosphorylation site domain) peptide synergi

259 % CI, 0.12-0.34, P < .001); CMR group vs the MPS group, 1.27 (95% CI, 0.79-2.03, P = .32).

260 Treatment with the MPS peptide suppressed levels of MARCKS phosphorylation

261 the protein CD64) were contained within the MPS cluster, no more distinct than other MPS cells.

262 abundance and regular distribution of tissue MPS cells, including novel macrophage populations within

263 irmed the restriction of CSF1R expression to MPS cells.

264 inflammatory-mediated mechanisms related to MPS diseases and perhaps lead to improved targeted thera

265 single-photon emission computed tomography (MPS).

266 families affected by dominantly transmitted MPS characterized by pterygia, camptodactyly of the hand

267 ents with chest pain who underwent treadmill MPS (n=4764), only 27% reported angina on the treadmill.

268 is of all MPS disorders except the ultrarare MPS-IX.

269 that the developmental mechanism underlying MPS differs from that of other conditions and/or that ce

270 our series there were several failures using MPSs as a strategy for rescue endotherapy suggesting tha

271 roup, 1.37 [95% CI, 0.52-3.57]; CMR group vs MPS group, 0.95 [95% CI, 0.46-1.95]).

272 longer-term (ingestion of deuterated water) MPS responses, the primary outcome measures.

273 ificantly diminished at the 46 th week, when MPS IIIB mice showed a major loss of rods and rod bipola

274 Critically, whereas MPS remained unchanged in the UT leg (e.g., approximatel

275 al and morphological changes associated with MPS IIIB disease progression using the established model

276 eneric coexpression clusters associated with MPS maturation and innate immune function.

277 Ten children with MPS-IH and >=2 years from successful HCT underwent IV-ER

278 ERT in previously transplanted children with MPS-IH appears safe and can reduce uGAG, although this i

279 Cytokine analyses in CSF of children with MPS-IH showed significantly elevated inflammatory marker

280 port of CSF characteristics in children with MPS-IH.

281 clinical and subclinical WHAE compared with MPS.

282 sative NAGLU variant in Schipperke dogs with MPS IIIB was identified and was found at high frequency

283 unrelated cell types that may interact with MPS cells in vivo.

284 27 consecutive MPS patients (8 patients with MPS I, 4 patients with MPS II, 9 patients with MPS IV, a

285 BMT increases the life span of patients with MPS IH, musculoskeletal manifestations are only minimall

286 ents (8 patients with MPS I, 4 patients with MPS II, 9 patients with MPS IV, and 6 patients with MPS

287 S I, 4 patients with MPS II, 9 patients with MPS IV, and 6 patients with MPS VI) and 20 normal contro

288 9 patients with MPS IV, and 6 patients with MPS VI) and 20 normal control subjects.

289 rs of the long-term outcome of patients with MPS-IH after successful HCT.

290 The long-term prognosis of patients with MPS-IH receiving HCT can be improved by reducing the age

291 Two hundred seventeen patients with MPS-IH successfully engrafted with a median follow-up ag

292 e majority of the transplanted patients with MPS-IH, with high variability between patients.

293 ated the CSF of 25 consecutive patients with MPS-IH.

294 es (6 patients) of 15 eyes (8 patients) with MPS I had narrow angles or peripheral iridocorneal touch

295 yes (3 patients) of 8 eyes (4 patients) with MPS II had plateau iris configuration, but all 8 eyes ha

296 All 18 eyes (9 patients) with MPS IV had normal angle structures, but 8 eyes (4 patien

297 es (5 patients) of 11 eyes (6 patients) with MPS VI had shallow but not closed angles (81.8%).

298 eover, the vertebral fusions in persons with MPS, coupled with evidence of MYH3 expression in bone, s

299 The phenotypic overlap among persons with MPS, coupled with physical findings distinct from other

300 ificant predictors of treatment failure with MPSs in refractory bile leaks.