ライフサイエンスコーパス: lysosomal enzyme

1 binding (Kd approximately 1 microm) toward a lysosomal enzyme.

2 etically modified to overexpress the missing lysosomal enzyme.

3 nly encode lysosomal proteins, most commonly lysosomal enzymes.

4 ers (LSDs) caused by deficiencies of soluble lysosomal enzymes.

5 ad to reduced stability of newly synthesized lysosomal enzymes.

6 CE (hUCE), which is involved in targeting of lysosomal enzymes.

7 mpanied by killing of T. gondii dependent on lysosomal enzymes.

8 ch generates mannose 6-phosphate residues on lysosomal enzymes.

9 sicular trafficking to avoid the toxicity of lysosomal enzymes.

10 correction of secondary elevations of other lysosomal enzymes.

11 ked preference for phosphodiester-containing lysosomal enzymes.

12 s an efficient carrier of Man-6-P-containing lysosomal enzymes.

13 N-glycans it encounters on newly synthesized lysosomal enzymes.

14 torage disorders rely on cross-correction of lysosomal enzymes.

15 the proteolytic processing of various other lysosomal enzymes.

16 ve secretory pathways, and in TGN sorting of lysosomal enzymes.

17 (GAGs) secondary to the absence of specific lysosomal enzymes.

18 nalysis in dried blood spots of steroids and lysosomal enzymes.

19 ma membrane marker recycling and delivery of lysosomal enzymes.

20 hyl ester) found on Dictyostelium discoideum lysosomal enzymes.

21 tors and partial mistargeting of a subset of lysosomal enzymes.

22 dependent on the autophagy protein ULK1 and lysosomal enzymes.

23 M106B deficiency causes reduction in several lysosomal enzymes.

24 diurnal profiles of levels and activities of lysosomal enzymes.

25 sphate targeting signal on newly synthesized lysosomal enzymes.

26 omains are involved in binding to individual lysosomal enzymes.

27 independent mannose 6-phosphate receptor for lysosomal enzymes.

28 lant lectin RTB as a novel carrier for human lysosomal enzymes.

29 sting that S. aureus perturbs acquisition of lysosomal enzymes.

30 livery, and effective tissue distribution of lysosomal enzymes.

31 ge of GUSB and secondary elevations of other lysosomal enzymes, a finding characteristic of lysosomal

32 in the gene encoding the glycogen-degrading lysosomal enzyme acid alpha -glucosidase (GAA) (also cal

33 Man-P-GlcNAc) were generated by treating the lysosomal enzyme acid alpha-glucosidase (GAA) with recom

34 e disorder (LSD) caused by deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA).

35 e's disease is caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase (GAA).

36 lic myopathy caused by the deficiency of the lysosomal enzyme acid alpha-glucosidase and results in c

37 mutations in the GBA1 gene that encodes the lysosomal enzyme acid beta-glucosidase (GCase).

38 Electron microscopy revealed that the lysosomal enzyme acid phosphatase colocalized with immun

39 In this study, we show that the lysosomal enzyme acid sphingomyelinase (ASM) is released

40 genetic deficiency of the glycogen-degrading lysosomal enzyme acid-alpha glucosidase.

41 Human lysosomal enzymes acid-beta-glucosidase (GCase) and acid

42 f proteins founded by the well characterized lysosomal enzyme, acid sphingomyelinase (ASMase).

43 Delivering therapeutic levels of lysosomal enzymes across the blood-brain barrier (BBB) h

44 Therefore, LLPL encodes a novel lysosomal enzyme, ACS.

45 The release of intracellular Ca2+ and lysosomal enzymes activated caspase-dependent apoptosis

46 We report for the first time that in s-IBM, lysosomal enzyme activities of cathepsin D and B were de

47 mal proteins in the liver, thereby impairing lysosomal enzyme activities.

48 antly alkalinized lysosomal pH and decreased lysosomal enzyme activities.

49 We also found decreased lysosomal enzyme activity and autophagic perturbations,

50 n lysosomal pH, which leads to impairment of lysosomal enzyme activity and disruption of autophagic p

51 hanges in phospho-ULK1 (Ser555), LC3-II, and lysosomal enzyme activity confirmed that adiponectin dir

52 t for lysosomal membrane destabilisation) on lysosomal enzymes activity and protein degradation, pork

53 iseases are characterized by deficiencies in lysosomal enzymes, allowing accumulation of target subst

54 Deficiency in the lysosomal enzyme alpha-galactosidase (alpha-GAL) causes

55 ramide (Gb(3)) caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-gal A).

56 fic treatment, caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-gal A).

57 orage disorder caused by a deficiency of the lysosomal enzyme alpha-galactosidase A (alpha-gal A).

58 y disease is caused by deficient activity of lysosomal enzyme alpha-galactosidase A.

59 type I (MPS I), a genetic deficiency of the lysosomal enzyme alpha-l-iduronidase (IDUA), exhibit acc

60 ulti-system disorder caused by deficiency of lysosomal enzyme alpha-L-iduronidase, and patients treat

61 The human lysosomal enzymes alpha-galactosidase (alpha-GAL, EC 3.2

62 Fusions of the human lysosomal enzymes alpha-l-iduronidase or acid alpha-gluc

63 The activities of the three lysosomal enzymes (alpha-glucosidase (AG), beta-galactos

64 A lysosomal enzyme, alpha-galactosidase A, was responsible

65 A lysosomal enzyme, alpha-L-iduronidase (IDUA), was used f

66 n megakaryocytic cells could overexpress the lysosomal enzyme, alpha-l-iduronidase (IDUA), which is d

67 gramming erythroid cells for production of a lysosomal enzyme, alpha-L-iduronidase (IDUA).

68 system, the DNA aptamer was conjugated to a lysosomal enzyme, alpha-l-iduronidase, from which mannos

69 In addition, the activities of three lysosomal enzymes analyzed were diminished in chlamydia-

70 e of generating and storing fully functional lysosomal enzymes and can also lead to efficient deliver

71 ficiency results in inefficient ER export of lysosomal enzymes and diminished levels of the enzymes a

72 I and MLIII mice exhibited reduced levels of lysosomal enzymes and enlarged lysosomes with abnormal s

73 y of the mutant receptor (E19Q/K137M) toward lysosomal enzymes and Man-6-P.

74 Numerous lysosomal enzymes and membrane proteins are essential fo

75 oteins, which are known to be substrates for lysosomal enzymes and play a role in regulating lysosome

76 (WT) decreases lysotracker signal, secreted lysosomal enzymes and SNAP23-mediated lysosome exocytosi

77 responsible for the anterograde transport of lysosomal enzymes and substrates.

78 LRP1 participates in cellular activation of lysosomal enzymes and through this mechanism, indirectly

79 ively stabilize GCase when compared to other lysosomal enzymes and to increase N370S mutant GCase pro

80 ntracellular protozoa must resist killing by lysosomal enzymes and toxic metabolites.

81 This review focuses on some of these lysosomal enzymes and transporters, as well as current t

82 ules had an acidic pH and normal activity of lysosomal enzymes and were positive for the proteins ess

83 tapasin and its sensitivity to inhibitors of lysosomal enzymes, and further distinguished by its depe

84 al strains, anti-fungal activity of purified lysosomal enzymes, and mechanisms of killing against C.

85 free, but not cell-associated, VZV, secreted lysosomal enzymes, and released infectious virions when

86 106b(-/-) mice, we show that, while multiple lysosomal enzymes are increased in Grn(-/-) brain at bot

87 Without this modification, lysosomal enzymes are missorted to the extracellular spa

88 Lysosomal enzymes are responsible for the degradation of

89 Lysosomal enzymes are synthesized in the endoplasmic ret

90 The majority of lysosomal enzymes are targeted to the lysosome by post-t

91 Lysosomal enzymes are targeted to the lysosome through b

92 ng peptides to promote brain delivery of the lysosomal enzyme arylsulfatase A (ASA).

93 ), a member of a large family of coregulated lysosomal enzymes, as a key regulator of Lyme-associated

94 mes of the lysosomal system), which recruits lysosomal enzymes at the ER to promote their Golgi trans

95 The CI-MPR recognizes lysosomal enzymes bearing the Man-6-P modification, whic

96 adation of glucosylceramide catalyzed by the lysosomal enzyme beta-glucocerebrosidase (GBA).

97 Mutations within the lysosomal enzyme beta-glucocerebrosidase (GC) result in

98 tions in the GBA gene, which encodes for the lysosomal enzyme beta-glucocerebrosidase (GCase), result

99 The lysosomal enzyme beta-glucuronidase (Gusb) is a key regu

100 forward genetics approach, we identified the lysosomal enzyme beta-glucuronidase (GUSB), a member of

101 The interaction affinity for the lysosomal enzyme beta-glucuronidase was also much lower

102 f affected uptake and biodistribution of the lysosomal enzyme beta-glucuronidase, the protein deficie

103 0 host genes examined by RNAi depletion, the lysosomal enzyme beta-hexosaminidase was identified as a

104 luding Man-6-P, pentamannosyl phosphate, the lysosomal enzyme, beta-glucuronidase, and the carbohydra

105 Binding affinity studies using the lysosomal enzyme, beta-glucuronidase, confirmed these re

106 >1000-fold decrease in the affinity for the lysosomal enzyme, beta-glucuronidase.

107 isorder in which a heritable deficiency of a lysosomal enzyme, beta-hexosaminidase, results in the st

108 LSDs mainly stem from deficiencies in lysosomal enzymes, but also in some non-enzymatic lysoso

109 models show that massive overexpression of a lysosomal enzyme can be associated with dramatic morphol

110 Lysosomal enzymes can be re-engineered for BBB transport

111 Lysosomal enzymes catalyze the breakdown of macromolecul

112 d protein response (UPR) kinase PERK and the lysosomal enzyme cathepsin B.

113 Elevated levels of the lysosomal enzyme cathepsin D are found in the early stag

114 Htt expression increased levels of the lysosomal enzyme cathepsin D by an autophagy-dependent p

115 ic vacuoles formed in vitro internalized the lysosomal enzyme cathepsin D in proportion to the polygl

116 al proteolysis and decreased activity of the lysosomal enzyme cathepsin D.

117 ously demonstrated a reduction in the plasma lysosomal enzyme, cathepsin D (CatD), in children with N

118 ysosomal genes and decreased activity of the lysosomal enzyme, cathepsin D.

119 Brain levels of the lysosomal enzymes cathepsins B and D were significantly

120 embranes, and defects in their catabolism by lysosomal enzymes cause a diverse array of diseases.

121 of the AP-3, retromer, and BLOC-1 complexes; lysosomal enzymes; CHC22; and five novel proteins of unk

122 Palmitoyl-protein thioesterase-1 (PPT1), a lysosomal enzyme, cleaves thioester linkages in S-acylat

123 Also, lysosomal enzyme concentrations were significantly incre

124 Most recombinant lysosomal enzymes contain oligosaccharides with both ter

125 intracellular transport of newly synthesized lysosomal enzymes containing mannose 6-phosphate on thei

126 ng approximately 60-fold higher affinity for lysosomal enzymes containing the phosphodiester Man-P-Gl

127 ced with a tissue-specific LV, can deliver a lysosomal enzyme continuously at supraphysiological leve

128 However, whether luminal lysosomal enzymes contribute to this process remains unk

129 The reduction of 11 lipid-degrading lysosomal enzymes correlated with reduced capacity for l

130 They show that beta-glucocerebrosidase-the lysosomal enzyme defective in patients with Gaucher dise

131 Lysosomal enzyme deficiencies comprise a large group of

132 tial therapeutic options for rare congenital lysosomal enzyme deficiencies, but enzymes in clinical u

133 constitutes an adaptable platform for other lysosomal enzyme deficiencies.

134 he central nervous system component of human lysosomal enzyme deficiencies.

135 e shown promising results for NCLs caused by lysosomal enzyme deficiencies.

136 pression of galactocerebrosidase (GALC), the lysosomal enzyme deficient in Krabbe disease.

137 However, lysosomal enzymes do not cross the blood-brain barrier (

138 fuscinosises: autofluorescent inclusions and lysosomal enzyme elevation.

139 ion of S-nitrosylation-resistant variants of lysosomal enzymes enhances autophagy, and pharmacologica

140 ulatory effects of BMP4 on the expression of lysosomal enzymes essential for osteoclastic bone resorp

141 e were smaller, had elevated levels of serum lysosomal enzymes, exhibited cartilage defects, and had

142 mphotoxin-alpha1beta2, and the cells had low lysosomal enzyme expression and retained opsonized antig

143 es, becomes more resistant to proteolysis by lysosomal enzymes from antigen-presenting cells such tha

144 le abnormalities, including hyposecretion of lysosomal enzymes from kidneys and depression of seroton

145 s unique ability to distinguish the 60 or so lysosomal enzymes from the numerous non-lysosomal glycop

146 receptors facilitate the delivery of nascent lysosomal enzymes from the trans-Golgi network to endoso

147 Lysosomal enzymes function optimally at low pH; as accum

148 ive disorder caused by the deficiency of the lysosomal enzyme galactocerebrosidase (GALC).

149 sease, which is affected by mutations in the lysosomal enzyme galactocerebrosidase, leading to the ac

150 The human lysosomal enzyme galactosamine-6-sulfatase (GALNS, also

151 ting disease caused by the deficiency of the lysosomal enzyme galactosylceramidase (GALC).

152 ating disorder caused by a deficiency in the lysosomal enzyme galactosylceramidase (GALC).

153 due to autosomal recessive mutations in the lysosomal enzyme galactosylceramidase (GALC).

154 y reported that mice deficient in UDP-GlcNAc:lysosomal enzyme GlcNAc-1-phosphotransferase (mucolipido

155 UDP-GlcNAc:lysosomal enzyme GlcNAc-1-phosphotransferase tags newly

156 Individuals with mutation in the lysosomal enzyme glucocerebrosidase (GBA) gene are at si

157 GBA encodes the lysosomal enzyme glucocerebrosidase (GCase) but the mech

158 Mutations in the GBA gene, which encodes the lysosomal enzyme glucocerebrosidase (GCase), are the mos

159 y mutations of the GBA gene that encodes the lysosomal enzyme glucocerebrosidase (GCase).

160 netic mutations within the gene encoding the lysosomal enzyme glucocerebrosidase (GCase).

161 in to saposin C, a critical activator of the lysosomal enzyme glucocerebrosidase (GCase).

162 used by mutations in GBA1, which encodes the lysosomal enzyme glucocerebrosidase (GCase).

163 utations in the GBA1 gene, which encodes the lysosomal enzyme glucocerebrosidase (GCase).

164 the lentivirus vector system to deliver the lysosomal enzyme glucocerebrosidase and a secreted form

165 Using the AAV-PHP.S capsid to target the lysosomal enzyme glucocerebrosidase for peripheral gene

166 link between mutations in GBA1, encoding the lysosomal enzyme glucocerebrosidase, and the synucleinop

167 The lysosomal enzyme glucocerebrosidase, encoded by the gluc

168 The lysosomal enzyme glucocerebrosidase-1 (GCase) catalyzes

169 rom an autosomal recessive deficiency of the lysosomal enzyme glucocerebrosidase.

170 dase 1 gene that result in deficiency of the lysosomal enzyme glucocerebrosidase.

171 n and consequently decreased activity of the lysosomal enzyme glucocerebrosidase.

172 ome inhibitor, but inhibitors of calpain and lysosomal enzymes had no effect.

173 red systemically, and the short half-life of lysosomal enzymes, hamper the development of effective t

174 The method was tested on a recombinant lysosomal enzyme, human alpha-galactosidase A, that cont

175 approach using CRISPR-Cas9 that targets the lysosomal enzyme iduronidase to the CCR5 safe harbor loc

176 beta-Glucuronidase (GUSB) is a lysosomal enzyme important in the normal step-wise degra

177 ntral nervous system (CNS), implicating this lysosomal enzyme in disease pathogenesis.

178 em mass spectrometry (MS/MS) based assays of lysosomal enzymes in dried blood spots for the early det

179 , to an understanding of the biochemistry of lysosomal enzymes in general, and to the cell biology of

180 In particular, the role of lysosomes and lysosomal enzymes in initiation and execution of the apo

181 ransferase is a key step in the targeting of lysosomal enzymes in mammalian cells and tissues.

182 derstood about the intracellular movement of lysosomal enzymes in neurons.

183 on (ERAD) prevents native folding of mutated lysosomal enzymes in patient-derived fibroblasts from tw

184 tigate the role of phosphodiester-containing lysosomal enzymes in the biogenesis of lysosomes.

185 f approximately 6), reducing the activity of lysosomal enzymes in the cells.

186 ysosome and impedes the activity of specific lysosomal enzymes indicating a broader role for chloride

187 ysosomal membranes to disruption, release of lysosomal enzymes into the cytosol, and neuronal degener

188 he data suggest that gD blocks the influx of lysosomal enzymes into the endosomal compartment by bind

189 ity, indicating that cathepsin D is the main lysosomal enzyme involved in alpha-synuclein degradation

190 staining for beta-hexosaminidase activity, a lysosomal enzyme involved in the degradation of GM2 gang

191 human alpha-mannosidase, MAN2B1, which is a lysosomal enzyme involved in the turnover of N-linked gl

192 s that result from the defective activity of lysosomal enzymes involved in glycosaminoglycan cataboli

193 Furthermore, non-lysosomal enzymes involved in the degradation of essenti

194 oses are caused by inherited deficiencies of lysosomal enzymes involved in the degradative pathway of

195 f infected macrophages and A. polyphaga, the lysosomal enzyme is present among the bacteria when host

196 Thus, secretion of lysosomal enzymes is a mycobactericidal mechanism that m

197 stablish that the human GAA gene, encoding a lysosomal enzyme, is a downstream target of the Notch-1/

198 In humans, loss of activity of a lysosomal enzyme leads to an inherited metabolic defect

199 s of lysosomal membrane integrity leading to lysosomal enzyme leakage into the cytoplasm.

200 Further, the increased lysosomal enzyme levels in lung of double mutant mice su

201 and Tmem106b genes have opposite effects on lysosomal enzyme levels, and their interaction determine

202 Such proteins include lysosomal enzymes, lysosomal integral membrane proteins,

203 es a flexible platform for the expression of lysosomal enzymes making it applicable to other lysosoma

204 The HPCD-mediated reduction of excess lysosomal enzymes may contribute to the ability of this

205 including FOXP2, CNTNAP2, ATP2C2, CMIP, and lysosomal enzymes, may advance our understanding of the

206 MLII mice showed lysosomal enzyme missorting and several skeletal alterat

207 AGLU mutation supports that some carriers of lysosomal enzyme mutations may develop later in life muc

208 caused by mutations in the gene encoding the lysosomal enzyme N-acetylgalactosamine-6-sulfate sulfata

209 UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferas

210 UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferas

211 uridine 5'-diphosphate-N-acetylglucosamine: lysosomal enzyme N-acetylglucosamine-1-phosphotransferas

212 UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferas

213 ridine diphosphate (UDP)-N-acetylglucosamine:lysosomal enzyme N-acetylglucosamine-1-phosphotransferas

214 nition of lysosomal hydrolases by UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferas

215 tion is catalyzed by UDP-N-acetylglucosamine:lysosomal enzyme N-acetylglucosamine-1-phosphotransferas

216 n of N-linked oligosaccharides by UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferas

217 ions in the alphabeta subunits of UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferas

218 nnose 6-phosphate is UDP-N-acetylglucosamine:lysosomal-enzyme-N-acetylglucosmine-1-phosphotrans feras

219 sine-based phosphorylation signals shared by lysosomal enzymes of diverse structure and function.

220 no mutant effects on levels or secretion of lysosomal enzymes of several other tissues.

221 typically a genetic deficiency of one of the lysosomal enzymes, often causing accumulation of undegra

222 he mannose 6-phosphate recognition marker on lysosomal enzyme oligosaccharides, resides primarily in

223 s pH-dependent; the homodimeric CD-MPR binds lysosomal enzymes optimally in the pH environment of the

224 ve internalized cells are either degraded by lysosomal enzymes or released.

225 heterogeneous disorders caused by defects in lysosomal enzymes or transporters, resulting in accumula

226 orage disorder caused by a deficiency of the lysosomal enzyme palmitoyl protein thioesterase 1 (PPT1)

227 utations in the Ppt1 gene, which encodes the lysosomal enzyme palmitoyl-protein thioesterase 1 (Ppt1)

228 Deficiency in a recently characterized lysosomal enzyme, palmitoyl-protein thioesterase (PPT),

229 CLN1 encodes a lysosomal enzyme, palmitoyl-protein thioesterase 1 (PPT1

230 A portion of the lysosomal enzymes produced by cells is secreted, diffuse

231 efective pigmentation, aberrant targeting of lysosomal enzymes, prolonged bleeding, and immunodeficie

232 a membrane sphingomyelin to ceramide by this lysosomal enzyme promotes lesion internalization.

233 Previously, we generated a lysosomal enzyme recognition domain in the secretory pro

234 can can serve as the minimal elements of the lysosomal enzyme recognition domain.

235 ication, decreased proteolytic processing of lysosomal enzymes, reduced degradation of lysosomal subs

236 tion with reduced adenosine triphosphate and lysosomal enzyme release.

237 ovide the most sensitive assay for the three lysosomal enzymes reported to date as shown by their per

238 age disorder caused by the deficiency of any lysosomal enzyme required for the breakdown of glycosami

239 galactosamine-6-sulfate sulfatase (GALNS), a lysosomal enzyme required for the stepwise degradation o

240 osaminoglycans (GAGs) due to deficiencies in lysosomal enzymes responsible for GAG breakdown.

241 class 3 (PIK3C3), Rab7, vacuolar ATPase, and lysosomal enzymes revealed that vacuole/lysosome fusion

242 ders rely on the receptor-mediated uptake of lysosomal enzymes secreted by cells, and for each lysoso

243 , they indicate that the GGAs participate in lysosomal enzyme sorting mediated by the CD-MPR.

244 ilarly impairs protein retrieval to the TGN, lysosomal enzyme sorting, endosomal cholesterol traffic

245 of GAK to the TGN and the function of GAK in lysosomal enzyme sorting.

246 ) in the Golgi and have an essential role in lysosomal enzyme sorting.

247 th mutations in this motif were defective in lysosomal enzyme sorting.

248 Purified lysosomal enzymes, specifically cathepsin B, inhibited c

249 CTSC is a regulator and activator of various lysosomal enzymes such as cathepsin B, D, and L.

250 ndent MPR (CD-MPR) are key components of the lysosomal enzyme targeting system that bind newly synthe

251 receptor (CD-MPR) is a key component of the lysosomal enzyme targeting system that binds newly synth

252 GGA2 binding to the CI-MPR is important for lysosomal enzyme targeting.

253 These data show that mutations in the lysosomal enzyme-targeting pathway produce highly specif

254 mmering, has been linked to mutations in the lysosomal enzyme-targeting pathway, but how this remarka

255 A lysosomal enzyme termed prenylcysteine lyase has been id

256 ort could occur, by expressing an eukaryotic lysosomal enzyme that can be visualized in tissue sectio

257 Acid alpha-glucosidase (GAA) is a lysosomal enzyme that degrades glycogen.

258 deletion of cGas in mice lacking DNaseII, a lysosomal enzyme that digests DNA, rescued the lethal au

259 al deficiency of glucocerebrosidase (GBA), a lysosomal enzyme that hydrolyzes glucosylceramide to cer

260 Acid alpha-glucosidase (GAA) is a lysosomal enzyme that hydrolyzes glycogen to glucose.

261 yelin phosphodiesterase, EC 3.1.4.12) is the lysosomal enzyme that hydrolyzes sphingomyelin (SPM) to

262 as for bound peptide Ag, the identity of the lysosomal enzyme that initiates invariant chain cleavage

263 ocerebrosidase (GBA) gene, which encodes the lysosomal enzyme that is deficient in Gaucher's disease,

264 deficient in lysosomal lipase 2 (lipl-2), a lysosomal enzyme that is transcriptionally up-regulated

265 on assisting the folding of mutant misfolded lysosomal enzymes that are otherwise degraded in ER-asso

266 efective maturation and excessive storage of lysosomal enzymes that are released upon platelet activa

267 ating the expression, import and activity of lysosomal enzymes that control the degradation of protei

268 rders that stem from the absence of specific lysosomal enzymes that degrade selected lipids.

269 e developing a technology for endocytosis of lysosomal enzymes that depends on generic, chemically co

270 The CI-MPR also recognizes lysosomal enzymes that elude UCE maturation and instead

271 f engulfed material is primarily mediated by lysosomal enzymes that function optimally within a narro

272 Degranulation of lysosomal enzymes that might be responsible for both fun

273 ndrome, MPS IIIA-D, results from deficits in lysosomal enzymes that specifically degrade heparan sulf

274 exploited for delivery of currently approved lysosomal enzyme therapeutics.

275 all molecules that bind and stabilize mutant lysosomal enzymes, thereby allowing proper cellular tran

276 tment whose leakiness blunts the toxicity of lysosomal enzymes, thereby increasing bacterial survival

277 ninvasive approach could deliver the missing lysosomal enzyme to a fetus with any lysosomal storage d

278 ted upon by an as yet unidentified endosomal/lysosomal enzyme to trigger fusion.

279 e receptors (MPRs) deliver newly synthesized lysosomal enzymes to endosomes and then recycle to the G

280 urements showed that the trafficking of some lysosomal enzymes to lysosomes was impaired.

281 This tag serves to direct the lysosomal enzymes to lysosomes.

282 Targeted delivery of lysosomal enzymes to the endocytic compartment of human

283 from endosomes to the Golgi after delivering lysosomal enzymes to the endocytic pathway.

284 (CD-MPR) plays a key role in the delivery of lysosomal enzymes to the lysosome by binding newly synth

285 eptor in targeting phosphodiester-containing lysosomal enzymes to the lysosome.

286 a novel method for targeting and delivery of lysosomal enzymes to their natural location: the endocyt

287 sphate receptor, with consequently disrupted lysosomal enzyme trafficking and abnormal lysosomal morp

288 endothelial transport as well as appropriate lysosomal enzyme trafficking and biological function.

289 ncodes a multifunctional protein involved in lysosomal enzyme trafficking, fetal organogenesis, tumor

290 LN2 disease is caused by a deficiency in the lysosomal enzyme tripeptidyl peptidase I, which results

291 ex as indicated by cleavage experiments with lysosomal enzymes (Tritosomes).

292 iseases is currently based on endocytosis of lysosomal enzymes via the mannose or mannose 6-phosphate

293 yst biogenesis, suggests that repurposing of lysosomal enzymes was an important step in the evolution

294 e same targeting reagents in wild-type mice, lysosomal enzymes were expressed that are deficient in F

295 In addition, measurements of serum lysosomal enzymes were performed.

296 Beta-mannosidase, a lysosomal enzyme which acts exclusively at the last step

297 glucosamine-1-phosphotransferase, which tags lysosomal enzymes with a mannose 6-phosphate marker for

298 Surface plasmon resonance analyses using lysosomal enzymes with defined N-glycans were performed

299 -1-phosphotransferase tags newly synthesized lysosomal enzymes with mannose 6-phosphate recognition m

300 mster ovary cells that enables production of lysosomal enzymes with N-glycans custom designed to affe