Báo cáo khoa học: Missense mutations as a cause of metachromatic leukodystrophy Degradation of arylsulfatase A in the endoplasmic reticulum potx

... which increases the lysosomal concentration of partially active GC vari- ants, as demonstrated by increased cellular GC activity, an increased concentration of lysosomal GC glyco- forms and increased ... (causing another lysosomal storage disease) were shown to be folding and trafficking mutants [16] before this was explored as a possibility in GD. Galactose administration increa...

Ngày tải lên: 18/02/2014, 16:20

... binding partner (stability- or affinity-based), one could divide a phage library in half and sort one half against a binding partner and the other half against an expression tag. A comparison of the ... constructing naı ¨ ve antibody libraries [15]. Here, the association of the variable heavy chain (V H )with protein A was used as a surrogate for direct stability meas...

Ngày tải lên: 19/02/2014, 12:20

... CP-pyk (5¢-ACGACTAGTGGATCCATNNNNNAGTTTATTCTT GACANNNNNNNNNNNNNNTGRTATAATNNNNAA GTAATAAAATATTCGGAGGAATTTTGAAATGAATA AACGTGTAAAAATCG-3¢)(N¼ A, T, G, C) and pyk- back (5¢-CTCTACATGCATTTCAACAATAGGGCCTG TC-3¢) ... (5¢-TGGTACTCGAG CAATTTCTGAAGGTATCGAAG-3¢) and pyk2 (5¢-GG AAGGATCCTTGTGTTTTTCTCCTATAATG-3¢) and downstream to pyk using primer pyk3 (5¢-GGAAGGA TCCTTTGTCAATTAATGATCTTAAAAC-3¢) and pyk4...

Ngày tải lên: 19/02/2014, 17:20

... that has them as their precondition. There may be multiple action instances in the plan that introduce the same atom canadjoin (A, u). In this case, we can freely choose one of these instances as ... A, u.¬mustadjoin (A, u). We can then send the actions and the initial state and goal specifications to any off -the- shelf planner and obtain the plan in Fig. 3. The straig...

Ngày tải lên: 20/02/2014, 12:20

... model. Linear discriminant analysis (LDA).Thisstatistical multivariate method is supervised. It searches for the variables containing the greatest interclass variance and the smallest intraclass variance, ... greatest part of the original data information. In the second part of our work, we focused on the biochemical information available i n infrared s pectra, and we found...

Ngày tải lên: 21/02/2014, 03:20

... further confirm if these variations in binding affinity were caused by binding instability as a result of nonspecific interference, rather than the alternation of a binding site, we carried out the ... AtERF4 was a repressor, an extra effector in which the activation domain of viral protein 16 was fused to the yeast GAL4 DNA binding domain (DBD) and then coexpressed wit...

Ngày tải lên: 18/02/2014, 13:20

... CYP 6A2 specific signal, the star indicates unspecific signal observed in all lanes loaded with bacterial protein. The CYP 6A2 variants have the same apparent molecularmassasCYP 6A2 fromD. melanogaster ... (CAGGACAGCCTGCGCAACGAG), RVR (CAG GACAGGGTGCGCAACGAG), SVR (CAGGACAG CGTGCGCAACGAG) and SLC (AGGGTATCCCTC TGCGATACG), respectively. All the alleles were inserted in pCW between t...

Ngày tải lên: 19/02/2014, 12:20

... token features from the first and last token in these paths. We also generate combination features by concatenating: (a) the last token in each path with the sequence of dependency labels along the ... level (since this is the “sentence” from the parser’s point of view). The majority of additional features that we introduced take advantage of the original text as...

Ngày tải lên: 20/02/2014, 04:20

... effect of HSS against CCCP-induced apoptosis, the activation of caspase-3 was examined using the enzymatic Caspase-Glo 3 ⁄ 7 assay. After CCCP treat- ment, caspase-3 activity increased markedly in ... characterized and the most prominent path- ways are the extrinsic and intrinsic pathways. In the intrinsic pathway (also known as the ‘mitochondrial pathway’), apoptosis re...

Ngày tải lên: 16/02/2014, 09:20

... 5¢-GGAGATCTCCAGTGA TATCGACCA-3¢ and 5¢-ACGGCTTCTACGGATCGAA ACT-3¢ for PPARc ,5¢-AAGACAGCTCCTCCTCGAAGG TT-3¢ and 5¢-TGACCAAATCCCCATTTACGC-3¢ for aP2, 5¢-ATCCATGGATGGACGGTAACG-3¢ and 5¢-CTGGA TCCCAATACTTCGACCA-3¢ ... 5¢-GCGTCGGGTAGATCCAGTT-3¢ and 5¢-CTC AGTGGGGCTTAGCTCTG-3¢ for ACC, and 5¢-AACAC CCCAGCCATGTACGTAG-3¢ and 5¢-TGTCAAAGAAA GGGTGTAAAACGC-3¢ for b-actin. Expression levels of the t...

Ngày tải lên: 16/02/2014, 09:20