Isolation of a branched chain alpha-keto acid dehydrogenase from rabbit liver. 1983

E Y Morrison, and K Mullings

An enzyme catalysing a series of reactions resulting in the oxidative decarboxylation of branched chain alpha-keto acids and production of NADH, was extracted from rabbit liver mitochondria with the aid of NaClO4. Purification yielded a product which appeared homogeneous on electrophoresis. The enzyme is active on three substrates alpha-ketoisocaproate, alpha-keto-beta-methyl valerate, and alpha-ketoisovalerate.

UI MeSH Term Description Entries
D007658 Ketone Oxidoreductases Oxidoreductases that are specific for KETONES. Oxidoreductases, Ketone
D008930 Mitochondria, Liver Mitochondria in hepatocytes. As in all mitochondria, there are an outer membrane and an inner membrane, together creating two separate mitochondrial compartments: the internal matrix space and a much narrower intermembrane space. In the liver mitochondrion, an estimated 67% of the total mitochondrial proteins is located in the matrix. (From Alberts et al., Molecular Biology of the Cell, 2d ed, p343-4) Liver Mitochondria,Liver Mitochondrion,Mitochondrion, Liver
D009097 Multienzyme Complexes Systems of enzymes which function sequentially by catalyzing consecutive reactions linked by common metabolic intermediates. They may involve simply a transfer of water molecules or hydrogen atoms and may be associated with large supramolecular structures such as MITOCHONDRIA or RIBOSOMES. Complexes, Multienzyme
D011817 Rabbits A burrowing plant-eating mammal with hind limbs that are longer than its fore limbs. It belongs to the family Leporidae of the order Lagomorpha, and in contrast to hares, possesses 22 instead of 24 pairs of chromosomes. Belgian Hare,New Zealand Rabbit,New Zealand Rabbits,New Zealand White Rabbit,Rabbit,Rabbit, Domestic,Chinchilla Rabbits,NZW Rabbits,New Zealand White Rabbits,Oryctolagus cuniculus,Chinchilla Rabbit,Domestic Rabbit,Domestic Rabbits,Hare, Belgian,NZW Rabbit,Rabbit, Chinchilla,Rabbit, NZW,Rabbit, New Zealand,Rabbits, Chinchilla,Rabbits, Domestic,Rabbits, NZW,Rabbits, New Zealand,Zealand Rabbit, New,Zealand Rabbits, New,cuniculus, Oryctolagus
D002417 Cattle Domesticated bovine animals of the genus Bos, usually kept on a farm or ranch and used for the production of meat or dairy products or for heavy labor. Beef Cow,Bos grunniens,Bos indicus,Bos indicus Cattle,Bos taurus,Cow,Cow, Domestic,Dairy Cow,Holstein Cow,Indicine Cattle,Taurine Cattle,Taurus Cattle,Yak,Zebu,Beef Cows,Bos indicus Cattles,Cattle, Bos indicus,Cattle, Indicine,Cattle, Taurine,Cattle, Taurus,Cattles, Bos indicus,Cattles, Indicine,Cattles, Taurine,Cattles, Taurus,Cow, Beef,Cow, Dairy,Cow, Holstein,Cows,Dairy Cows,Domestic Cow,Domestic Cows,Indicine Cattles,Taurine Cattles,Taurus Cattles,Yaks,Zebus
D000818 Animals Unicellular or multicellular, heterotrophic organisms, that have sensation and the power of voluntary movement. Under the older five kingdom paradigm, Animalia was one of the kingdoms. Under the modern three domain model, Animalia represents one of the many groups in the domain EUKARYOTA. Animal,Metazoa,Animalia
D013045 Species Specificity The restriction of a characteristic behavior, anatomical structure or physical system, such as immune response; metabolic response, or gene or gene variant to the members of one species. It refers to that property which differentiates one species from another but it is also used for phylogenetic levels higher or lower than the species. Species Specificities,Specificities, Species,Specificity, Species
D013293 Enterococcus faecalis A species of gram-positive, coccoid bacteria commonly isolated from clinical specimens and the human intestinal tract. Most strains are nonhemolytic. Streptococcus Group D,Streptococcus faecalis
D042942 3-Methyl-2-Oxobutanoate Dehydrogenase (Lipoamide) A ketone oxidoreductase that catalyzes the overall conversion of alpha-keto acids to ACYL-CoA and CO2. The enzyme requires THIAMINE DIPHOSPHATE as a cofactor. Defects in genes that code for subunits of the enzyme are a cause of MAPLE SYRUP URINE DISEASE. The enzyme was formerly classified as EC 1.2.4.3. 2-Oxoisovalerate Dehydrogenase (Lipoamide),2-Oxoisocaproate Dehydrogenase,Alpha-Keto Acid Dehydrogenase,BCKA Decarboxylase,Branched Chain Alpha-Keto Acid Decarboxylase,Branched Chain Ketoacid Dehydrogenase,Branched-Chain 2-Oxo Acid Dehydrogenase,Branched-Chain Keto Acid Dehydrogenase,Branched-Chain Oxo-Acid Dehydrogenase,2 Oxoisocaproate Dehydrogenase,Acid Dehydrogenase, Alpha-Keto,Alpha Keto Acid Dehydrogenase,Branched Chain 2 Oxo Acid Dehydrogenase,Branched Chain Alpha Keto Acid Decarboxylase,Branched Chain Keto Acid Dehydrogenase,Branched Chain Oxo Acid Dehydrogenase,Decarboxylase, BCKA,Dehydrogenase, 2-Oxoisocaproate,Dehydrogenase, Alpha-Keto Acid,Dehydrogenase, Branched-Chain Oxo-Acid,Oxo-Acid Dehydrogenase, Branched-Chain
D051381 Rats The common name for the genus Rattus. Rattus,Rats, Laboratory,Rats, Norway,Rattus norvegicus,Laboratory Rat,Laboratory Rats,Norway Rat,Norway Rats,Rat,Rat, Laboratory,Rat, Norway,norvegicus, Rattus

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