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 ally colocalizes with cellular endosomal and lysosomal proteins.

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

8 ompanied by increased expression of specific lysosomal proteins.

9 and we reveal a significant upregulation of lysosomal proteins.

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

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

12 orders caused by mutations of genes encoding lysosomal proteins.

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

14 ranslational modification and trafficking of lysosomal proteins.

15 s resulted from a failure to dephosphorylate lysosomal proteins.

16 gression, and numerous induced genes encoded lysosomal proteins.

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

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

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

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

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

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

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

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

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

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

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

28 Most lysosomal proteins are tagged with a carbohydrate modifi

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

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

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

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

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

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

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

36 Inhibition of lysosomal protein degradation by bafilomycin or chloroqu

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

62 Lysosomal protein LAMP-1 that was exocytosed during degr

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

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

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

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

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

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

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

70 Studies of lysosomal proteins linked to neurodegenerative disorders

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

72 idenced by acidification and accumulation of lysosomal protein markers.

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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

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