From analysis of cDNA clones, they found that the collagenous Gly-Xaa-Yaa repeat sequence has 5 'imperfections' that coincide with those in the corresponding portion of the alpha-1 IV chain. Antiserum against an alpha-5 IV synthetic peptide stained a polypeptide chain of about kD by immunoblot analysis.
Immunolocalization in the human kidney was almost completely restricted to the glomerulus. The calculated molecular mass of the mature chain isDa. They determined the sequence of the 19 3-prime-most exons and their flanking sequences from genomic phage clones, with the exception of exon 15, which was sequenced after amplification from genomic DNA by PCR.
The exon sizes in the 3-prime half of the gene bear a remarkable similarity to those observed in the alpha-1 IV chain. High-resolution PFGE mapping with the rare-cutting enzyme XhoI showed that the gene is at least kb long, one of the largest collagen genes characterized to date.
Using a zebrafish model, Xiao et al. The features vary from the severe clinically and genetically heterogeneous renal disorder Alport syndrome see, e. The authors stated that more than different mutations had been identified in the COL4A5 gene.
The spectrum of mutations was broad and provided insight into the clinical variability of Alport syndrome with respect to age at renal failure and accompanying features. However, Barker et al. The deletions were dispersed all over the gene with different sizes ranging from 1 kb to the complete absence of the gene more than kb in 1 patient.
In 4 patients with intragenic deletions, absence of the alpha-3 IV chain in the glomerular basement membrane was demonstrated by immunohistochemical methods. This finding supported the hypothesis that abnormalities of the alpha-5 IV chain may prevent normal incorporation of the alpha-3 IV chain into the glomerular basement membrane.
Direct sequencing of cDNA amplified from lymphoblast mRNA of 4 patients with internal gene deletions, using appropriate combinations of primers amplifying across the predicted boundaries of the deletions, allowed Antignac et al.
Regardless of the extent of deletion and of the putative protein product, the 14 deletions occurred in patients with juvenile-type Alport syndrome. They noted that intronic changes such as inversions shown to be pathogenic in hemophilia; see and Naylor et al.
A partial explanation for this was suggested by the findings of King et al. One mutation created a new donor splice site within an intron resulting in the inclusion of a novel in-frame cryptic exon; a second mutation resulted in a new exon splice enhancer sequence ESE that promoted splicing of a cryptic exon containing a stop codon; a third mutation was a base substitution within the polypyrimidine tract that precedes the acceptor splice site that resulted in exon skipping.
All 3 cases would have been missed using an exon-by-exon DNA screening approach.Zara 7S Framework its architecture will determine the path that the company will go through to reach their goals. Many ways to do this kind of analysis exist and are used world widely, but the ones that are .
As some of you may know, I am writing a book on operating models, which will probably be called “Operating Model Canvas” – to connect with the “Business Model Canvas”.
I am also making this the center piece of my course Designing Operating Models.. So here is something I have been working on for the book – the eleven visuals.
|Account Options||Skills Shared Values Strategy is a plan developed by a firm to achieve sustained competitive advantage and successfully compete in the market.|
|Of course, once my book on operating models — Operating Model Canvas — is available in March, IT leaders can use it instead! The issue we were debating is whether a reference model should have one value chain or many and if many — how many.|
|Style Staff "Hard" elements are easier to define or identify and management can directly influence them:|
3 AUGUST ZARA McKinsey 7S Model ON THE COVER 3 MARCH ZARA RECOMMENDATIONS Recommendation 1 ON THE COVER 3 MARCH ZARA RECOMMENDATIONS ON THE COVER 3 MARCH ZARA ON THE COVER 3 AUGUST ZARA STRATEGY Zara's Organisational Strategy Being an international company Zara has adopted .
Zara's business model June 23, / Alexander Osterwalder Go for a shopping trip to one of H&M 's clothing stores and then compare with one of the shops of the Spanish chain Zara.
While some models of organizational effectiveness go in and out of fashion, one that has persisted is the McKinsey 7-S framework. Search the world's information, including webpages, images, videos and more. Google has many special features to help you find exactly what you're looking for.