Krebs cycle diseases

The biochemical classification of mitochondrial DNA is based on the five major steps of mitochondrial metabolism. These steps are illustrated in Figure 42-3 and divide mitochondrial diseases into five groups defects of mitochondrial transport, defects of substrate utilization, defects of the Krebs cycle, defects of the respiratory chain and defects of oxidation-phosphorylation coupling. 

Defects of the Krebs cycle. Fumarase deficiency was reported in children with mitochondrial encephalomyop-athy. Usually, there is developmental delay since early infancy, microcephaly, hypotonia and cerebral atrophy, with death in infancy or early childhood. The laboratory hallmark of the disease is the excretion of large amounts of fumaric acid and, to a lesser extent, succinic acid in the urine. The enzyme defect has been found in muscle, liver and cultured skin fibroblasts [16]. 

In 1954 Krebs was appointed the Whitley Chair of Biochemistry at Oxford University. That same year he received the Royal Medal of the Royal Society of London. In 1958, for his scientific work and his contributions to the lives of British people, Krebs was knighted. Even after his retirement in 1967, he continued to do research on fiver disease, the genetic bases of metabolic diseases, and the link between poor nutrition and juvenile delinquency. In addition to his Nobel Prize and Royal Medal, he received honorary degrees ftom nine universities, see also Glycolysis Krebs Cycle. 

Rustin P, Bourgeron T, Parfait B, Chretien D, Munnich A, Rotig A. Inborn errors of the Krebs cycle a group of unusual mitochondrial diseases in human. Biochim Bio-phys Acta 1997 1361(2) 185-197... 

In 1966 Fischer and Krebs published their seminal work on protein phosphorylation and its regulatory function in cellular pathways.1 Since then, great strides have been made in our understanding of protein kinases, their functional roles in cellular processes, and their pathogenic roles in diseases. Bishop and Varmus received the 1989 Nobel Prize for their work on oncogenic kinases.2 In 2001, Nurse and Hunt were also awarded the Nobel Prize for their work in elucidating the role of cyclins and cyclin-dependent kinases in regulating cell cycles.3... 

---