ライフサイエンスコーパス: cystatin B

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1 eletion of the lysosomal protease inhibitor, cystatin B.

2 s mouse model of EPM1 provides evidence that cystatin B, a non-caspase cysteine protease inhibitor, h

3 mutations in the gene (CSTB) encoding human cystatin B, a widely expressed cysteine protease inhibit

4 y form of cerebral amyloid angiopathy whilst cystatin B aggregates are found in cases of Unverricht-L

5 Here we apply limited proteolysis to cystatin B amyloid fibrils and show that not only the al

6 Previous work on cystatin B amyloid fibrils revealed that the alpha-helic

7 mined the structured core of human stefin B (cystatin B) amyloid fibrils using quenched hydrogen exch

8 ied the effects of diminishing expression of cystatin B, an endogenous inhibitor of cathepsins B, H a

9 over in TgCRND8 mice by genetically deleting cystatin B, an endogenous inhibitor of lysosomal cystein

10 s of two major cysteine protease inhibitors, cystatin B and C, were unchanged.

11 eine protease inhibitors (CPI), stefin A and cystatins B and C, by these same venules.

12 evidence that mutations in the gene encoding cystatin B are responsible for the primary defect in pat

13 ed to the cytosol, where the CTSB inhibitors cystatin-B/C were abundantly present.

14 Loss-of-function mutations in the cystatin B (Cstb) gene cause a neurological disorder kno

15 and three were successfully verified, namely cystatin B (CSTB), triosephosphate isomerase (TPI1), and

16 Loss-of-function mutations in the cystatin B (CysB) gene, an intracellular cysteine protea

17 Cystatin B deletion in TgCRND8 significantly reduces the

18 Cystatin B deletion rescued autophagic-lysosomal patholo

19 We found that mice lacking cystatin B develop myoclonic seizures and ataxia, simila

20 Inherited mutations in the cystatin B gene ( CSTB ) are responsible for progressive

21 tation, were identified in the gene encoding cystatin B in EPM1 patients but were not present in unaf

22 We show that decreased expression of cystatin B in patient fibroblasts enhances cathepsin act

23 of lipid accumulation in TgCRND8 by removing cystatin B inhibition on lysosomal proteases suggests th

24 Stefin B (cystatin B) is an endogenous cysteine cathepsin inhibito

25 with available data for amyloids from either cystatin B or cystatin C.

26 m ROS was attenuated in an NPC cell model by cystatin B over-expression or pharmacological inhibition

27 Cystatin B was recently identified as an acid-resistant

28 The gene encoding cystatin B was shown to be localized to this region, and

29 In addition, cathepsin H and cystatin B were colocalized in type 2 alveolar epithelia

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