ライフサイエンスコーパス: limp

1 t dimers and test candidate explanations for limping.

2 or the origin of kinesin step asymmetry and "limping."

3 Enterovirus 71 also binds to LIMP-2 (also known as SCARB2) on the external surface of

4 ucocerbrosidase (GCase)) binding sequence to LIMP-2 (lysosomal integral membrane protein 2), the rece

5 Heterologous expression of LIMP-2 accelerated clearance of overexpressed alpha-synu

6 Depletion of LIMP-2 alters SREBP-2-mediated cholesterol regulation, a

7 n at any of these decreases GCase binding to LIMP-2 and alters its pH-dependent binding as well as di

8 a similar affinity to that observed between LIMP-2 and beta-GCase.

9 ino acid, but the interactions of GCase with LIMP-2 are heavily influenced by Asp(399) and the di-iso

10 These data support a role for LIMP-2 as the mannose-6-phosphate-independent traffickin

11 Remarkably, we find that LIMP-2 bears P-Man9GlcNAc2 covalently attached to residu

12 l interface region within GC as critical for LIMP-2 binding and lysosomal transport.

13 e report structural results illuminating how LIMP-2 binds and releases beta-GCase according to change

14 how that the cavity in the luminal domain of LIMP-2 can bind and deliver exogenous cholesterol to the

15 LIMP-2 deficiency is associated with neurological abnorm

16 a demonstrated that the crystal structure of LIMP-2 displays a hydrophobic three-helix bundle compose

17 LIMP-2 expression also led to lysosomal transport of a b

18 Therefore, we suggest that manipulating LIMP-2 expression to increase lysosomal GC activity is a

19 In cellular uptake experiments, LIMP-2 facilitates transport of phospholipids into murin

20 basis and functional importance of a form of LIMP-2 for lipid trafficking.

21 Binding of these lipids alters LIMP-2 from functioning as a glucocerebrosidase-binding

22 o be responsible for controlling the exit of LIMP-2 from the Golgi.

23 Variants in the LIMP-2 gene cause action myoclonus-renal failure syndrom

24 The integral membrane protein LIMP-2 has been a paradigm for mannose 6-phosphate recep

25 Based on these findings, we generated a LIMP-2 helix 5-derived peptide that precipitated and act

26 and cell-based assays that support a role of LIMP-2 in cholesterol transport.

27 Given the importance of GC and LIMP-2 in disease pathogenesis, we studied their interac

28 olgi compartment, leading to accumulation of LIMP-2 in enlarged endosomal vesicles.

29 Reconstitution of LIMP-2 in LIMP-2-deficient fibroblasts led to a rescue o

30 hosphate in the Golgi caused accumulation of LIMP-2 in this compartment, and PI4KIIIbeta was found to

31 imaging microscopy, we also demonstrate that LIMP-2 interacts with MPR in living cells.

32 that the lysosomal integral membrane protein LIMP-2 is a specific binding partner of beta-glucocerebr

33 The binding to LIMP-2 is not dependent upon a single amino acid, but th

34 ormation of cholesterol(-)like inclusions in LIMP-2 knockout mice suggested the possibility that LIMP

35 n surviving DA neurons of human PD midbrain, LIMP-2 levels were increased, probably to compensate for

36 via a histidine trigger, and suggesting that LIMP-2 localizes the ceramide portion of the substrate a

37 Here we report a crystal structure of a LIMP-2 luminal domain dimer with bound cholesterol and p

38 Understanding the interplay of GCase and LIMP-2 on a structural level is crucial to identify pote

39 Our data indicate that LIMP-2 operates in parallel with Niemann Pick (NPC)-prot

40 ron microscopy with the aid of an engineered LIMP-2 shuttle and two GCase-targeted pro-macrobodies.

41 The detection of cholesterol within the LIMP-2 structure and the formation of cholesterol(-)like

42 g between monomeric and dimeric forms allows LIMP-2 to engage distinct binding partners, a mechanism

43 We identify helix 5 and helix 7 of LIMP-2 to interact with a binding pocket in GCase, formi

44 s study, we resolve the complex of GCase and LIMP-2 using cryo-electron microscopy with the aid of an

45 resulted in their rapid degradation, whereas Limp-2 was relatively stable in the lysosome in the abse

46 lysosomal integral membrane protein type-2 (LIMP-2) as a proprietary lysosomal transporter.

47 lysosomal integral membrane protein type-2 (LIMP-2) plays a pivotal role in the delivery of beta-glu

48 lysosomal integral membrane protein type-2 (LIMP-2), we studied alpha-synuclein metabolism in LIMP-2

49 lysosomal integral membrane protein type 2 (LIMP-2).

50 lysosomal integral membrane protein type 2 (LIMP-2, also known as SCARB2) reveals a large cavity tha

51 These results revise the accepted view of LIMP-2-beta-GCase lysosomal targeting.

52 In LIMP-2-deficient brains a significant reduction in GC ac

53 Reconstitution of LIMP-2 in LIMP-2-deficient fibroblasts led to a rescue of beta-glu

54 evels were significantly higher in sera from LIMP-2-deficient mice compared to wild-type.

55 2), we studied alpha-synuclein metabolism in LIMP-2-deficient mice.

56 nd protein levels were severely decreased in LIMP-2-deficient mouse tissues.

57 uced alpha-synuclein levels, suggesting that LIMP-2-derived peptides can be used to activate endogeno

58 enger receptor proteins highly homologous to LIMP-2.

59 s interaction with the transmembrane protein LIMP-2.

60 iple steps in the trafficking pathway of the LIMP-2/GBA complex.

61 the medium, which was attenuated by limiting LIMP-2/GBA exit from the Golgi by PI4KIIIbeta inhibitors

62 data also provide a structural model of the LIMP-2/GC complex that will facilitate the development o

63 However, the LIMP-2/SCARB2 binding sequences for enterovirus 71 and G

64 1 and GCase are not similar, indicating that LIMP-2/SCARB2 may have multiple or overlapping binding s

65 Lysosomal integral membrane protein-2 (LIMP-2/SCARB2) contributes to endosomal and lysosomal fu

66 ol transporters, NPC1 and to a lesser extent LIMP-2/SCARB2, bind to sphingosine and showed that their

67 n sequential steps, causing these motors to "limp" along the microtubule.

68 itioning were combined, the rats developed a limp and a tilted posture that correlated in direction a

69 The bulk elastic properties of limp and aging cellular solids are calculated for model

70 in stalk stiffness, ruling out models where limping arises from an asymmetry in torsional strain.

71 stigation of young children who present with limping as their only or predominant symptom.

72 Defect-only rabbits limped at all times.

73 The mechanics of an aged, limp beam is calculated, thus offering a practical proce

74 Limping behavior implies that the molecular rearrangemen

75 how that the experimentally observed kinesin limping can be explained in our model by the variation o

76 GFP tagging of LIMP caused a limping defect during movement with reduce

77 GFP tagging of LIMP caused a limping defect during movement with reduced speed and tr

78 ment in limp, indicated by the percentage of limp graded as none and mild to the total, was much high

79 tructs with short stalks have been found to "limp", i.e., exhibit alternation in the dwell times of s

80 ctivity of the glycine motif-deficient SR-BI/LIMP II chimera was low but could be increased by introd

81 The rate of lipid uptake mediated by SR-BI/LIMP II chimeras was proportional to the extent of recep

82 acking (lysosomal integral membrane protein (LIMP) II) this glycine motif (chimeras).

83 LCMV was greatly enhanced by addition of the LIMP-II tail.

84 pe was almost abolished when attached to the LIMP-II tail.

85 l of lysosomal integral membrane protein-II (LIMP-II).

86 Limping implies that kinesin molecules strictly alternat

87 ne of the most common causes of hip pain and limp in young children.

88 elatively common conditions that can produce limping in children 1-6 yr old.

89 Satisfactory improvement in limp, indicated by the percentage of limp graded as none

90 LIMP is an essential motility and invasion factor necess

91 Transcribed in gametocytes, LIMP is translated in the ookinete from maternal mRNA, a

92 p abductor and flexor strength, and enhanced limp recovery without an increased risk in complications

93 gth, radiographic review, complications, and limp recovery.

94 The absence of LIMP reduces initial mosquito infection by 50%, impedes

95 chanism by which such rearrangements lead to limping remains unsolved.

96 stalk region near the heads had no effect on limping, ruling out possible stalk misregistration durin

97 fusion protein and five out of 10 developed limp tails.

98 integral membrane proteins (termed Lamps and Limps) that are extensively glycosylated with asparagine

99 Here, we identify the Plasmodium protein LIMP (the name refers to a gliding phenotype in the spor

100 However, limping was enhanced by perturbations that increased the

101 Limping was equally unaffected by mutations that produce