Neonatal diabetes mellitus is a rare form of Insulin dependent diabetes mellitus that present Within the first month of life, lasting at least two weeks and requiring insulin therapy. Intrauterine growth restriction, failure to thrive, fever, dehydration, hyperglycemia and acidosis with or without ketonuria are the clinical features of the disease. Monogenic forms of diabetes account for about 1 to 5 percent of all cases of diabetes in young people. In most cases of monogenic diabetes, the gene mutation is inherited; in the remaining cases the gene mutation develops spontaneously. Most mutations in monogenic diabetes reduce the body's ability to produce insulin, a protein produced in the pancreas that helps the body use glucose for energy. Neonatal diabetes mellitus (NDM) and maturity-onset diabetes of the young (MODY) are the two main forms of monogenic diabetes. MODY is much more common than NDM. NDM first occurs in newborns and young infants; MODY usually first occurs in children or adolescents but may be mild and not detected until adulthood . Micro satellites are known to be highly polymorphic due to the high rate of mutations in their tracts (Fan H , Chu, J.Y 2007). These mutations can be either in the form of increase / decrease of repeat units or in the form of single nucleotide substitutions/ deletions/insertions and other events (Li, Y.C., Korol, A.B., Fahima, T. and Nevo, E. 2004) . Increase or decrease of repeat units of micro satellites in coding regions might lead to shift in reading frames there by causing changes in protein product (Martin P 2005)and in non-coding regions are known to effect the gene regulation(Sibly 2003 et al). Point mutations (Substitutions and Indels) are also found to occur at a higher rate in micro satellites than elsewhere (Stenson 2003 et al). Micro satellite mutations with in or near certain genes are known to be responsible for some human neurodegenerative diseases. So, we made a brief study to check whether the mutations in this KCNJ11 gene have any relation with these micro satellites repeats and the study revealed the following results.

All the 30 proved mutations except the mutations, which occur at codon numbers 12,23,34 and, 35 of the KCNJ11gene are falling inside the coding region and are eventually leading to phenotypic differences were collected from the Human Gene Mutation Database (HGMD)(Mudunuri S.B., Nagarajaram 2007). Micro satellites are obtained from the Imperfect Micro satellite Extractor (IMEX) (Letunic, I., et al 2004)tool using intermediate mode with default values 10 for single 5 for di 3 tri 3 for tetra 2 for penta and 2 for hexa and obtained only 4 micro satellites in KCN1J1. Since micro satellites are drawn from the nucleotide sequence and HGMD mutations are given for protein sequence we have used DNA to Amino Acid translator. We compared the microsatellite regions with the mutations whether they have mutations in those regions and found that no mutations fall in the microsatellite regions. Now we analyzed whether these mutations have fallen in the functional domains of those genes by using Simple Modular Architecture Research Tool (SMART)( Endocr Dev 2007) and the results are as follows.

The microsatellites found in KCNJ11 gene and the mutations of KCNJ11 gene are.

The mutations in the KCNJ11 are causing the neonatal diabetes mellitus. These mutations result in reduced ATP sensitivity of theKATP channels compared with the wild types. The level of channel activity defect is responsible for different clinical features: the 'mild' form confers isolated permanent neonatal diabetes whereas the severe form combines diabetes and neurological symptoms such as epilepsy, developmental delay, muscle weakness and mild dysmorphic features.so to check whether is there any relationship is there between the microsatellites and the mutations. we analyzed and found that there are no mutations in the microsatellite regions and therefore can say that the microsatellites are not responsible for mutations in the KCNJ11 gene.