THE MITOCHONDRIAL SUGAR DISEASE AND MOLECULAR MECHANISM

Raghavendra Rao M. V, Abrar A. Khan, Mohammed Khaleel, Khizer Hussain Junaidy, Amreen Hamza, Mahendra Kumar Verma, Kumar Ponnusamy, Dorababu P, Dilip Mathai

Abstract


Mitochondria are the power factories of the cells and produce ATP by oxidizing reducing equivalents via the respiratory chain. 

These reducing equivalents originate mainly from the citric acid cycle that also occurs within the mitochondria. Human mitochondria contain their own genetic material in the form of circular DNA that encodes for only a fraction of the mitochondrial components. The other mitochondrial components are nuclear encoded.

Furthermore, the respiratory chain and mitochondrionassociated monoamine oxidase are major producers of reactive oxygen radicals. As a result, mutations in mtDNA can deregulate multiple processes within cells and the balance of this deregulation may contribute to the clinical phenotype  Mitochondrial diabetes affects up to 1% of patients with diabetes and is often unrecognized by the physicians.

Diabetes and deafness (DAD) or maternally inherited diabetes and deafness (MIDD) or mitochondrial diabetes is a subtype of diabetes which is caused from a point mutation at position 3243 in human mitochondrial DNA, which consists of a circular genome. This affects the gene encoding tRNALeu. Diabetes mellitus is a common feature of mitochondrial disease, but rarely occurs in isolation; other clinical features depend largely on the underlying mtDNA or nuclear DNA mutation. 

Because mitochondrial DNA is contributed to the embryo by the oocyte and not by spermatozoa, this disease is inherited from maternal family members only. As indicated by the name, MIDD is characterized by diabetes and sensori neural hearing loss. As suggested by the name, patients with MIDD are subject to sensorineural hearing loss. MIDD has also been associated with a number of other issues including kidney dysfunction, gastrointestinal problems, and cardiomyopathy  


Keywords


Diabetes and deafness (DAD) , Sensorineural hearing loss.Cardiomyopathy , Adenosine deaminase, Glucokinase gene in type 2 diabetes mellitus, Maternally inherited diabetes and deafness (MIDD) ,Retinal vascular disease , Gluconeogenesis Introduction MtD

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