The below mentioned article provides a study note on the Prenatal Diagnosis of Genetic Diseases.

It is required to analyse DNA of family members of the affected individual to set up a diagnostic tool for a genetic disease. The markers (RFLPs) that are tightly linked to the disease is needed to be identi­fied first.

The physicians are required to have a ba­sic understanding of the application of DNA tech­nology to diagnosis of genetic diseases in the in­terest of medical and legal reasons. In case of fami­lies with a history of severe genetic diseases, it is essential to determine the presence of the disorder in the developing fetus.

Methods Available for Prenatal Diagnosis:

1. The fetus should be visualized by ultra­sound if the genetic abnormality is caused in gross anatomic defects.

2. The chemical composition of the amni­otic fluid can also give diagnostic clues.

3. Molecular analysis of fetal DNA can pro­vide the most detailed genetic picture per­mitting reproductive information.

Sources of DNA:

DNA is obtained from white blood cells, amniotic fluid or chorionic villi. The time for DNA analysis has been shortened by the development of PCR.

Diagnosis of Sickle Cell Diseases Using Recombinant DNA Technology:

1. In the past, prenatal diagnosis of hemoglobinopathies was involved in the determination of the amount and kinds of hemoglobin synthesized in red cells ob­tained from fetal blood.

2. Sickle cell anemia is a genetic disease caused by a point mutation. At present, diagnostic techniques for analysing fetal DNA has become valuable since they pro­vide safe, early detection of genetic dis­eases.

Phenylketonuria:

1. The phenylalanine hydroxylase (PAH) gene has been located on chromosome 12. The presence of abnormal phenylalanine hydroxylase genes has been detected by the use of DNA polymorphism as markers to distinguish between normal and mu­tant genes.

2. If an unborn fetus is affected, DNA-based screening is useful not only in determin­ing but also in detecting carriers of the PKU gene. PKU is inherited as an auto­somal recessive trait. It is also important to identify heterozygotes for future fam­ily planning.

Probes:

1. Probes are generally pieces of DNA or RN A labelled with a p32-containing nucleotide.

2. The probe must know a complementary sequence to be effective.

3. A cDNA synthesized from a specific mRNA can be used to screen either a cDN A library for a longer cDNA or a genomic library for a complementary sequence in the coding region of a gene.

4. cDNA probes are used to detect DNA frag­ments on southern blot transfers and to detect and quantitate RNA on Northern blot transfers.

Blotting and Hybridization Techniques:

1. Observation of a specific DNA or RNA fragment in many “Contaminating” mol­ecules requires a number of techniques which are collectively termed blot trans­fer.

2. Sometimes, if a specific base is altered and a restriction site is changed, these proce­dures can detect a point mutation.

3. The Northern and Western blot transfer techniques are used to size and quantitate specific RNA and protein molecules, re­spectively.

4. Colony or plaque hybridization is the method by which specific clones are iden­tified and purified. Bacteria are grown on colonies on an agar plate and overlaid with a nitrocellulose filter paper.

5. Perfect matches hybridize readily and with­stand high temperatures in the hybridiza­tion and washing reactions. These com­plexes also form in the presence of low salt concentrations.

6. Less than perfect matches do not tolerate these stringent conditions.

7. Gene families can be detected by varying the stringency of the hybridization and washing steps.

DNA Sequencing:

1. The segments of specific DNA molecules can be analysed for their nucleotide se­quence. This method depends upon hav­ing a large number of identical DNA mol­ecules.

2. This requirement can be satisfied by clon­ing the fragment of interest by the above techniques.

3. Enzymatic method uses specific deoxy-nucleotide analogs that terminate DNA strand synthesis at specific nucleotides as the strand is synthesized on purified tem­plate nucleic acid.

Manual and Automatic Techniques for the Determination of the Sequence of DNA:

1. The segments of specific DNA molecules obtained by recombinant DNA technol­ogy can be analysed for their nucleotide sequence. The manual, emymatie method (Sanger’s) employs specific dideoxynucleotides that terminate DNA strand syn­thesis at specific nucleotides.

2. Polyaerylamitte gel electrophoresis can be used to separate the fragments according to size. Each of the fragments produces an image (band) on an x-ray film.

3. Another manual method, that of Maxam and Gilbert employs chemical methods to cleave the DNA molecules where they contain the specific nucleotides.

4. The most commonly procedure is the four different fluorescent labels, each repre­senting one nucleotide. Each emits a spe­cific signal by a laser beam and this can be recorded by a computer.