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p to now, all of the chapters have concentrated on the process of utilizing information within the Perl environment and the Perl script you have invoked However, it is often desirable, if not essential, to create, update, and access information for external databases This process is called object persistence, since the data is created and is persistently (or permanently) available There are many different types of database systems Some of them will be familiar to many of you, as you probably already use them, although you may not always realize that you are doing so Most databases take one of two basic forms: flat file and relational With a flat-file database, the information is stored in a fixed format, and the information stored is considered to be isolated or complete For example, the /etc/passwd file under Unix or an ini file under Windows could both be considered examples of a flat-file database The information is stored in its entirety in a format that is easy to understand, and it does not need to be linked to another database for the information to make sense A relational database, on the other hand, uses a number of separate tables Each table contains a list of information, and links between the tables enable you to store information in a structured and relative way For example, imagine a music database It might have two tables one lists the artists and the other lists the individual recordings they have made You can access all of the recordings for a particular artist by examining the links between the two tables When it comes to accessing and using databases, there are other problems to contend with First of all, you must decide how you are going to store the information within a file Using a single- or multicharacter delimiter is a fairly common system and one that Unix uses in many different places By using a tab or colon or some other character, you can separate the individual fields within a record The return character is often used to separate the individual records within the entire file The problem with this method is that if you want to store information that may possibly contain one of these delimiting characters, you may experience some corruption of data or even complete loss of information A better solution in this instance is to use a fixed-length record system Rather than delimiting the individual fields and records, fixed-length systems specify the length of each field Providing you know the format (field sizes and types), you should be able to read entire records from the file and determine the individual fields by their positions This is wasteful of space for databases with very large fields In a database with 16K records, you can easily eat up a lot of space A record with 1K of useful data still takes up 16K of storage space Even once you have solved these problems, there are other considerations Text databases are great for accessing sequential information or for storing a stream of information that will be processed by a program at a later time What they are not so good at is random access Searching through a large text database can be a time-consuming task, since you will need to read in each individual record to determine whether it is the one you want There are ways around this: you can use an index system to point to the records you want, but the chances are that this also uses a text format, and you end up back at square one.

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13:

This category includes a quartet of human diseases so-called Creutzfeldt-Jakob disease (and a variant that infects cows and may be rarely transmitted to humans), the Gerstmann-Straussler Scheinker syndrome, kuru, and probably, fatal familial insomnia Although this group of diseases has been included for discussion in the chapter on viral diseases of the nervous system, it has been evident for some time that the cause of these slow infections is neither a virus nor a viroid (nucleic acid alone, without a capsid structure) During the past 20 years, Prusiner is credited with doggedly pursuing this problem, for which he was awarded the Nobel prize He and his colleagues have presented compelling evidence that the transmissible pathogen is a proteinaceous infectious particle that is devoid of nucleic acid, resists the action of enzymes that destroy RNA and DNA, fails to produce an immune response, and electron microscopically does not have the structure of a virus To distinguish this pathogen from viruses and viroids, Prusiner introduced the term prion The prion protein (PrP) is encoded by a gene on the short arm of chromosome 20 The discovery of mutations in the PrP genes of patients with familial Creutzfeldt-Jakob disease and Gerstmann-Straussler-Scheinker syndrome (described below) attests to the fact that prion diseases are both genetic and infectious In this respect, prions are unique among all infectious pathogens It is now possible to detect inherited types of prion disease during life, using DNA extracted from leukocytes How prions arise in sporadic forms of spongiform encephalopathy is not fully understood; probably the conversion of the normal cellular protein to the infectious form involves a conformational change in the protein structure as described in Prusiner s review in 2001 What is remarkable is the current theory that abnormally folded prion protein can act as a template for the conversion of normal PrP to PrPsc (the latter denoting the scrapie prion; see further on) A description of the human prion diseases follows, the most important by far being Creutzfeldt-Jakob disease.

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