ライフサイエンスコーパス: lysosomal protein

1 interaction with the RILP (Rab7-interacting lysosomal protein).

2 ible genes and encodes an integral membrane, lysosomal protein.

3 onally processed into a approximately 40-kDa lysosomal protein.

4 of Transmembrane Protein 106B (TMEM106B), a lysosomal protein.

5 ction with the Rab7 effector Rab-interacting lysosomal protein.

6 Man-6-P glycoproteins that may represent new lysosomal proteins.

7 deficiency increases the cellular levels of lysosomal proteins.

8 ally colocalizes with cellular endosomal and lysosomal proteins.

9 and to glycosylation sites on the surface of lysosomal proteins.

10 tates and cosediments with mitochondrial and lysosomal proteins.

11 cluding Abeta-binding proteins and autophagy/lysosomal proteins.

12 ocytosis with upregulation of CD68 and Lamp1 lysosomal proteins.

13 at were enclosed within EVs enriched in endo-lysosomal proteins.

14 ompanied by increased expression of specific lysosomal proteins.

15 favored the modification of smaller, soluble lysosomal proteins.

16 and we reveal a significant upregulation of lysosomal proteins.

17 roteins involved in RNA translation and some lysosomal proteins.

18 for the F box protein Saf1 were all vacuolar/lysosomal proteins.

19 se 6-phosphate recognition marker on soluble lysosomal proteins.

20 orders caused by mutations of genes encoding lysosomal proteins.

21 CS-internalized CFTR did not colocalize with lysosomal proteins.

22 ranslational modification and trafficking of lysosomal proteins.

23 s resulted from a failure to dephosphorylate lysosomal proteins.

24 gression, and numerous induced genes encoded lysosomal proteins.

25 sease-associated risk gene(1) that encodes a lysosomal protein(2,3) that is highly enriched in axonal

26 lections of images of the distributions of a lysosomal protein, a Golgi protein, and nuclear DNA, the

27 Endogenous transferrin receptor and lysosomal proteins accumulated at the cell surface upon

28 Results showed several DC lysosomal proteins affected the growth of C. neoformans

29 We observed differential changes in lysosomal proteins among symptomatic mutation carriers w

30 Since saposin C is a lysosomal protein and pH gradients occur in lysosomes, w

31 ated induction of TFEB caused an increase in lysosomal protein and the lysosomal abundance in cell.

32 lysosomes, defined by accumulation of three lysosomal proteins and an acidophilic dye.

33 on, CSPalpha aggregates, increased levels of lysosomal proteins and lysosome enzyme activities.

34 620N] mutation alters the expression of ~220 lysosomal proteins and stimulates recruitment and phosph

35 sis that triggers the vesicular secretion of lysosomal proteins and the clathrin adaptor AP-2 mu2.

36 idues on the surface of cathepsins and other lysosomal proteins are a shared component of the recogni

37 Lysosomal proteins are also enriched at the PM in GM1 ga

38 In each case, some soluble vacuolar/lysosomal proteins are believed to be bound by transmemb

39 Most newly synthesized soluble lysosomal proteins are delivered to the lysosome via the

40 vity was high in the liver, where endogenous lysosomal proteins are efficiently dephosphorylated, but

41 Most lysosomal proteins are tagged with a carbohydrate modifi

42 main of the TGN from which newly synthesized lysosomal proteins are targeted to the late endosomes an

43 Despite being a lysosomal protein, ARSA directly interacts with alpha-sy

44 show that reduction of progranulin (PGRN), a lysosomal protein associated with TDP-43 proteinopathy,

45 Loss-of-function mutations in a lysosomal protein, ATP13A2 (PARK9), cause Kufor-Rakeb sy

46 ed stability of the TFEB protein and altered lysosomal protein biosynthesis.

47 bition, and blunting of cathepsin-L-mediated lysosomal protein breakdown.

48 ation on rat brain, EPDR co-distributes with lysosomal proteins, but there is significant overlap bet

49 ting that PIKfyve modulates the abundance of lysosomal proteins by affecting the degradation of these

50 Finally, we identified nine novel lysosomal proteins by using the CLEAR network as a tool

51 Among these, 28 soluble lysosomal proteins catalyzing the degradation of various

52 al axis involves suppression of proapoptotic lysosomal protein cathepsin D by promotion of the ER-ass

53 Although the lysosomal protein CD63 was mislocalized to the plasma me

54 ne in a manner requiring RAG-GTPases and the lysosomal protein complex Ragulator, but that this proce

55 Inhibition of lysosomal protein degradation by bafilomycin or chloroqu

56 Given the importance of autophagy and lysosomal protein degradation for cellular proteostasis

57 We identified lysosomal protein degradation of ADA2 as a pathomechanis

58 Autophagy is a conserved lysosomal protein degradation pathway whose precise role

59 Here we show how selective and non-selective lysosomal protein degradation pathways cooperate to ensu

60 are transitory, linking the proteasomal and lysosomal protein degradation pathways.

61 iated autophagy (CMA) is a selective form of lysosomal protein degradation that, in response to diver

62 ry cultures or human iPS neurons compromises lysosomal protein degradation, causes accumulation of al

63 ebrosidase (GBA) are known to interfere with lysosomal protein degradation, GBA heterozygotes may dem

64 ated autophagy (CMA)(5), a selective form of lysosomal protein degradation, is involved in sustaining

65 However, inhibition of autophagy or lysosomal protein degradation, while having no effect on

66 fficient to cause a significant reduction in lysosomal protein degradation.

67 disrupts axonal projections through impaired lysosomal protein degradation.

68 nd apoptosis in MEFs but ultimately impaired lysosomal protein degradation.

69 rone-mediated autophagy (CMA) is a selective lysosomal protein degradative process that is activated

70 g expression of the progranulin and TMEM106B lysosomal proteins, did not alter the somatic tau inclus

71 ources for understanding the contribution of lysosomal protein dynamics to signal transduction, organ

72 ed with anti-human endothelium NOS, anti-rat lysosomal protein (ED1), or anti-rat major histocompatib

73 However, the route taken by the endosomal/lysosomal protein endolyn-78 partially resembled the tra

74 We demonstrate that despite upregulation of lysosomal protein expression by constitutive activation

75 Antibodies to several different lysosomal proteins failed to label the chlamydial vacuol

76 anulin interactions with prosaposin, another lysosomal protein, first occur within the lumen of the e

77 insight into the relationship between these lysosomal proteins, GCase, and SNCA, and reveal novel th

78 These data show that many DC lysosomal proteins have antifungal activity and have pot

79 Mutations in many lysosomal proteins have catastrophic effects and cause n

80 sociated with protein synthesis/degradation (lysosomal proteins, heat shock proteins, and proteasomes

81 we propose a new network uniting three major lysosomal proteins: (i) cathepsin D (CTSD), which plays

82 deficiency in HE1, a ubiquitously expressed lysosomal protein identified previously as a cholesterol

83 CLN3 encodes a transmembrane lysosomal protein implicated in autophagy, endosomal tra

84 type C2 (NPC2) protein is a small, soluble, lysosomal protein important for cholesterol and sphingol

85 mbined with an analysis of the expression of lysosomal proteins in different tissues, led us to ident

86 tic synaptic vesicle proteins and known AP-3 lysosomal proteins in isolated synaptic vesicle fraction

87 plasmic reticulum, Golgi, late endosomal, or lysosomal proteins in the inclusion.

88 ate that obesity promotes S-nitrosylation of lysosomal proteins in the liver, thereby impairing lysos

89 termates, and western blots showed increased lysosomal proteins including LAMP-2, cathepsin D and LC3

90 easome activity and an increase in autophagy-lysosomal proteins, including LC3-II and LAMP1.

91 fibroblasts revealed extensive missorting of lysosomal proteins, including sphingolipid catabolism en

92 lysosomes in a mutant mouse lacking NPC2, a lysosomal protein involved in lipid transport.

93 P-3 subunits, synaptic vesicle proteins, and lysosomal proteins known to be sorted in an AP-3-depende

94 Lysosomal protein LAMP-1 that was exocytosed during degr

95 ecognized the tyrosine signal from the human lysosomal protein LAMP-2, HTGYEQF.

96 isolectin IB(4), and stain intensely for the lysosomal proteins Lamp-1, Lamp-2, and cathepsin D as we

97 and YopD colocalized with the late endosomal/lysosomal protein LAMP1 and that the frequency of YopD a

98 fy a subset of astrocytes that expresses the lysosomal protein LAMP1(2) and the death receptor ligand

99 mTOR and the lysosomal protein LAMP2 were highly co-localised in basa

100 OR; lysosome size; number; pH; expression of lysosomal proteins LAMP2, P62, and LC3B; and lysosomal f

101 m106b deletion from Grn(-/-) mice normalizes lysosomal protein levels and rescues FTLD-related behavi

102 ring lysosomal acidification and normalizing lysosomal protein levels in Grn(-/-) neurons.

103 ab effector-related proteins Rab-interacting lysosomal protein-like 1 (Rilpl1) and Rilpl2 regulate pr

104 Studies of lysosomal proteins linked to neurodegenerative disorders

105 Expression of the lysosomal protein, lysosomal-associated membrane protein

106 fluid requires processing that involves two lysosomal proteins, lysosomal acid lipase, which hydroly

107 alectins and ubiquitylation of transmembrane lysosomal proteins, many steps in the process, and their

108 idenced by acidification and accumulation of lysosomal protein markers.

109 e to lysosomal impairment, overproduction of lysosomal proteins may also contribute to FTD pathogenes

110 (MFSD8) gene, which encodes a membrane-bound lysosomal protein, MFSD8.

111 nherited defects in genes that mainly encode lysosomal proteins, most commonly lysosomal enzymes.

112 Saposin C is a lysosomal protein needed for optimal GCase activity.

113 NPC) disease, where defects in the endosomal-lysosomal protein NPC1 or NPC2 cause intracellular accum

114 c screens, we highlight the discovery of the lysosomal proteins NPC1 and LAMP1 as intracellular recep

115 al storage disorder caused by defects in the lysosomal proteins NPC1 or NPC2.

116 d by defects in either of two late endosomal/lysosomal proteins, NPC1 and NPC2.

117 Niemann-Pick C1 (NPC1), a lysosomal protein of 13 transmembrane helices (TMs) and

118 thin the CLN3 gene, which encodes a putative lysosomal protein of unknown function, are the underlyin

119 ytosis causing inappropriate accumulation of lysosomal proteins on the cell surface.

120 levels of alpha-synuclein and key autophagy/lysosomal proteins over time in the exogenous alpha-synu

121 Here we demonstrate that Tip targets a lysosomal protein p80, which consists of an N-terminal W

122 ived cholesterol from lysosomes requires two lysosomal proteins, polytopic membrane-bound Niemann-Pic

123 is lost in iPSC-derived neurons lacking the lysosomal protein progranulin.

124 Unexpectedly, knockdown of the lysosomal protein prosaposin strongly sensitizes neurons

125 ons, we identified Psap, a gene encoding the lysosomal protein prosaposin, as closely related with mT

126 is required for the efficient sorting of the lysosomal protein prosaposin.

127 depleted of Niemann-Pick disease type C1, a lysosomal protein required for cholesterol extraction fr

128 We identify a gene, psidin, that encodes a lysosomal protein required in the blood cells for both d

129 ermeable type 2 two-pore channels (TPC2) are lysosomal proteins required for nicotinic acid adenine d

130 r NPC2, which are membrane-bound and soluble lysosomal proteins, respectively.

131 n host cells deficient for late endosomal or lysosomal proteins revealed that the Niemann-Pick type C

132 Rab7-interacting lysosomal protein (RILP) and oxysterol-binding protein-r

133 -endosomal transport by the Rab7-interacting lysosomal protein (RILP) is reversed by LIC1 RNAi, which

134 o test if the dynein adapter RAB-interacting lysosomal protein (RILP) mediated the recruitment of dyn

135 in decreased recruitment of Rab-interacting lysosomal protein (RILP), an effector that regulates the

136 ding to SPDL1, BICD2, HOOK3, RAB-interacting lysosomal protein (RILP), RAB11 family-interacting prote

137 nding to mammalian-specific RAB7 interacting lysosomal protein (RILP).

138 ls, which do not efficiently dephosphorylate lysosomal proteins, significantly decreased the steady s

139 al membrane, two essential steps in vacuolar/lysosomal protein sorting from yeast to humans.

140 oteins are known to participate in endosomal/lysosomal protein sorting in higher eukaryotes.

141 The involvement of various lysosomal proteins, such as TFEB, TMEM175, GBA, and LAMP

142 ce brains lowered levels of ATG proteins and lysosomal proteins, suggesting its role in the regulatio

143 sociated proteins, transcription factors and lysosomal proteins suggests the need for co-regulation o

144 tations in arrestin, and by elimination of a lysosomal protein, Sunglasses.

145 -MIM2 interactions inhibit VPS4 recruitment, lysosomal protein targeting, and HIV-1 budding.

146 onal analyses indicate that MFSD12 encodes a lysosomal protein that affects melanogenesis in zebrafis

147 lphaVbeta5 and with RAB7 and Rab-interacting lysosomal protein that are required for phagosome matura

148 abeling, we show that NPC2, a late endosomal/lysosomal protein that binds to cholesterol with high af

149 NPC2 is a soluble lysosomal protein that functions in coordination with NP

150 agy modulator), a p53 target gene encoding a lysosomal protein that induces macroautophagy, as an eff

151 Here, we show PGRN is a secretory lysosomal protein that regulates lysosomal function and

152 mbrane 4 L six family member 19 (TM4SF19), a lysosomal protein that represses acidification through i

153 Recent work has identified a lysosomal protein that transports neutral amino acids (L

154 ) belongs to a small group of non- enzymatic lysosomal proteins that act as cofactors in the sequenti

155 This prompted us to look for lysosomal proteins that are involved in lal(-/-) EC dysf

156 and validation of both luminal and membrane lysosomal proteins that should be applicable to high thr

157 ress of cholesterol from the lysosomes and 2 lysosomal proteins, the NPC1 (Niemann-Pick C1) and NPC2

158 In addition to lysosomal proteins, these organelles contain cell type-s

159 DRAM2 encodes a transmembrane lysosomal protein thought to play a role in the initiati

160 Loss of COMMD3 increased the release of lysosomal proteins through extracellular vesicles, leadi

161 , which exhibited increased routing of these lysosomal proteins through the plasma membrane.

162 By contrast, the lysosomal protein TMEM106B appeared unique to SARS-CoV-2

163 Here we show that the lysosomal protein TMEM55B contributes to restore cellula

164 unclear whether mTORC1 phosphorylates local lysosomal proteins to regulate specific aspects of lysos

165 n is recognized by receptors that target the lysosomal proteins to the lysosome where, in most cell t

166 exposed to an acidic environment translocate lysosomal proteins to their surface, thus protecting the

167 t indicate that VipA, VipD, and VipF inhibit lysosomal protein trafficking by different mechanisms; o

168 In more evolved cells, lysosomal protein trafficking is achieved by cargo recog

169 Genetic, immunofluorescence microscopy, and lysosomal protein trafficking studies indicate that the

170 ed VPS-41, a multidomain protein involved in lysosomal protein trafficking, as a modifier of alpha-sy

171 oteins (Vac1p, Vps27p) required for vacuolar/lysosomal protein trafficking.

172 phosphate-binding domains, where it disrupts lysosomal protein trafficking.

173 OGT, and OGA at the GATA-binding site of the lysosomal protein transmembrane 5 (Laptm5) gene promoter

174 ide signaling at distinct stages of vacuolar/lysosomal protein transport and couple PtdIns(3,5)P2 syn

175 To study the role of carbohydrate in lysosomal protein transport, we engineered two novel gly

176 the multilamellar interior of late endosomal/lysosomal proteins, ultimately effecting cholesterol egr

177 which exhibit increased trafficking of this lysosomal protein via the plasma membrane.

178 Protein expression of GCase and other lysosomal proteins was determined by western blotting.

179 beta-galactosidase, a lysosomal protein, was elevated 3.6-5.7-fold significant

180 significant alterations to trafficking of a lysosomal protein were observed in the procyclic stage,

181 In liver, mRNAs encoding several lysosomal proteins were elevated, including NPC1 and NPC

182 Upon GLA modRNA treatment, a subset of lysosomal proteins were partially restored to wild-type

183 , as well as antibodies to late endosomal or lysosomal proteins, were used in conjunction with confoc

184 TRPML1 and TRPML2 homomultimers are lysosomal proteins, whereas TRPML3 homomultimers are in

185 e Rab7 adaptor protein RILP (Rab interacting lysosomal protein), which is responsible for linking Rab

186 omal enzymes, but also in some non-enzymatic lysosomal proteins, which lead to abnormal storage of ma

187 Here we show that NKG7, a lysosomal protein whose expression is restricted to cyto

188 Progranulin is a lysosomal protein whose haploinsufficiency causes fronto

189 CLN5 is a soluble lysosomal protein with an unclear function in the cell.

190 al transmembrane protein and NPC2, a soluble lysosomal protein with cholesterol binding properties.

191 cDNA led to the production of a glycosylated lysosomal protein with palmitoyl-CoA hydrolase activity

192 gi, a phosphotransferase specifically labels lysosomal proteins with mannose 6-phosphate (Man-6-P).

193 Expression of genes encoding lysosomal proteins with roles in sphingolipid metabolism

194 of PeMP phagocytic cups, the localization of lysosomal proteins within them, and phagocytosis of apop