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 ked preference for phosphodiester-containing lysosomal enzymes.

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

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

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

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

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

9 ma membrane marker recycling and delivery of lysosomal enzymes.

10 hyl ester) found on Dictyostelium discoideum lysosomal enzymes.

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

12 diurnal profiles of levels and activities of lysosomal enzymes.

13 ontributes to high affinity interaction with lysosomal enzymes.

14 he mannose 6-phosphate recognition signal on lysosomal enzymes.

15 formation of mannose 6-phosphate residues on lysosomal enzymes.

16 teract with the highly diverse population of lysosomal enzymes.

17 h factor beta, and regulate the targeting of lysosomal enzymes.

18 A in control of intracellular trafficking of lysosomal enzymes.

19 inity exhibited by the CD-MPR toward various lysosomal enzymes.

20 sphate targeting signal on newly synthesized lysosomal enzymes.

21 omains are involved in binding to individual lysosomal enzymes.

22 independent mannose 6-phosphate receptor for lysosomal enzymes.

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

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

25 livery, and effective tissue distribution of lysosomal enzymes.

26 nly encode lysosomal proteins, most commonly lysosomal enzymes.

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

28 ad to reduced stability of newly synthesized lysosomal enzymes.

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

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

31 M106B deficiency causes reduction in several lysosomal enzymes.

32 sicular trafficking to avoid the toxicity of lysosomal enzymes.

33 correction of secondary elevations of other lysosomal enzymes.

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

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

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

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

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

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

40 II), which is caused by a deficiency of the lysosomal enzyme acid alpha-glucosidase.

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

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

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

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

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

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

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

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

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

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

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

52 antly alkalinized lysosomal pH and decreased lysosomal enzyme activities.

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

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

55 e, continuous, low-level expression of intra-lysosomal enzyme activity in the brain can preserve CNS

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

77 ndrome that leads to increased extracellular lysosomal enzymes and might lead to tissue damage and co

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

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

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

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

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

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

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

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

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

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

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

89 106b(-/-) mice, we show that, while multiple lysosomal enzymes are increased in Grn(-/-) brain at bot

90 Lysosomal enzymes are responsible for the degradation of

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

92 Lysosomal enzymes are targeted to the lysosome through b

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

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

95 t mediates both the intracellular sorting of lysosomal enzymes bearing mannose 6-phosphate (Man-6-P)

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

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

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

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 le increased levels of the polymorphonuclear lysosomal enzyme, beta-glucuronidase, in GCF were associ

107 >1000-fold decrease in the affinity for the lysosomal enzyme, beta-glucuronidase.

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

109 ytosis, fluid phase efflux, and secretion of lysosomal enzymes but are unexpectedly more efficient in

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

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

112 Lysosomal enzymes catalyze the breakdown of macromolecul

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

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

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

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

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

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

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

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

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

122 of metabolism due to deficiencies of soluble lysosomal enzymes cause global neurodegenerative disease

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

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

125 Lysosomal enzyme concentrations are significantly elevat

126 Also, lysosomal enzyme concentrations were significantly incre

127 Most recombinant lysosomal enzymes contain oligosaccharides with both ter

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

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

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

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

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

133 Experimental correction of lysosomal enzyme deficiencies in animal models suggests

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

135 e expanded to include the treatment of other lysosomal enzyme disorders.

136 nged bleeding time, pigment dilution, kidney lysosomal enzyme elevation, serum alpha1-antitrypsin act

137 fuscinosises: autofluorescent inclusions and lysosomal enzyme elevation.

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

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

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

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

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

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

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

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

146 Lysosomal enzymes function optimally at low pH; as accum

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

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

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

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

151 acid alpha-glucosidase gene as well as other lysosomal enzyme genes.

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

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

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

155 caused by mutations in the gene encoding the lysosomal enzyme glucocerebrosidase (GC).

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

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

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

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

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

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

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

163 The lysosomal enzyme glucocerebrosidase, encoded by the gluc

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

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

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

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

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

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

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

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

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

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

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

175 condary changes in activity of several other lysosomal enzymes in liver and brain and elevation of ga

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

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

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

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

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

181 We have measured seven lysosomal enzymes in the plasma of 15 patients with Lowe

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

183 Pretreatment with lysosomal enzyme inhibitors [(2S, 3S)trans-epoxysuccinyl

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

210 ea influence the interaction with UDP-GlcNAc:lysosomal enzyme N-acetylglucosamine-1-phosphotransferas

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

231 tion with reduced adenosine triphosphate and lysosomal enzyme release.

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

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

234 U) encoding alpha-N-acetylglucosaminidase, a lysosomal enzyme required for the stepwise degradation o

235 se (N-acylsphingosine amidohydrolase) is the lysosomal enzyme required to hydrolyze the N-acyl linkag

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

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

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

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

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

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

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

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

244 Purified lysosomal enzymes, specifically cathepsin B, inhibited c

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

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

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

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

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

250 A lysosomal enzyme termed prenylcysteine lyase has been id

251 A lysosomal enzyme termed prenylcysteine lyase has been id

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

253 Acid ceramidase (AC) is the lysosomal enzyme that degrades ceramide into sphingosine

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

255 se, the product of a housekeeping gene, is a lysosomal enzyme that degrades glycogen.

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

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

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

259 n thioesterase-1 (PPT1) is a newly described lysosomal enzyme that hydrolyzes long chain fatty acids

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

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

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

263 n palmitoyl-protein thioesterase 1 (PPT1), a lysosomal enzyme that removes fatty acyl groups from cys

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

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

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

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

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

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

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

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

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

273 In addition to the intracellular sorting of lysosomal enzymes, the mannose 6-phosphate/insulin-like

274 hyl ester) found on Dictyostelium discoideum lysosomal enzymes: the amino-terminal CRD binds mannose

275 exploited for delivery of currently approved lysosomal enzyme therapeutics.

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

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

278 6-phosphate receptor (IGF2R) interacts with lysosomal enzymes through two binding domains in its ext

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

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

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

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

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

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

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

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

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 omes, we postulate that there is a defect in lysosomal enzyme trafficking in patients with Lowe syndr

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

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

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

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

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

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

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

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

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

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

300 -1-phosphotransferase tags newly synthesized lysosomal enzymes with mannose 6-phosphate recognition m