Results 91 to 100 of about 80,661 (193)

Mapping of the l-methylmalonyl-CoA mutase gene to mouse chromosome 17

Genomics, 1990
In humans, methylmalonyl acidemia is caused by a deficiency of L-methylmalonyl-CoA mutase (MUT) controlled by a gene that has been mapped to chromosome 6. The mouse homolog of this gene has now been mapped to mouse chromosome 17. Recombinant inbred and congenic strains place the mouse Mut locus 1.06 cM distal to H-2, between Pgk-2 and Ce-2.
J, Sertić, V, Vincek, F D, Ledley, F, Figueroa, J, Klein   +4 more
openaire   +2 more sources

Computational Insights into the Mechanism of Radical Generation in B₁₂-Dependent Methylmalonyl-CoA Mutase

, 2016
ONIOM calculations have provided novel insights into the mechanism of homolytic Co−C5‘ bond cleavage in the 5‘-deoxyadenosylcobalamin cofactor catalyzed by methylmalonyl-CoA mutase.
Ilja V. Khavrutskii (138382), Djamaladdin G. Musaev (1391992), Ruma Banerjee (45954), Piotr Paneth (808019), Renata A. Kwiecien (2653012), Keiji Morokuma (1247166)   +5 more
core   +1 more source

Mitochondrial dysfunction drives a neuronal exhaustion phenotype in methylmalonic aciduria

Communications Biology
Methylmalonic aciduria (MMA) is an inborn error of metabolism resulting in loss of function of the enzyme methylmalonyl-CoA mutase (MMUT). Despite acute and persistent neurological symptoms, the pathogenesis of MMA in the central nervous system is poorly
Matthew C. S. Denley, Monique S. Straub, Giulio Marcionelli, Miriam A. Güra, David Penton, Igor Delvendahl, Martin Poms, Beata Vekeriotaite, Sarah Cherkaoui, Federica Conte, Ferdinand von Meyenn, D. Sean Froese, Matthias R. Baumgartner   +12 more
doaj   +1 more source

methylmalonyl CoA racemase reaction

, 1997
In the metabolism of propionyl CoA, D-methylmalonyl CoA is produced by a carboxylase reaction. This product must be converted to L-methylmalonyl CoA in order to be metabolized further.

core  

Direct Infusion Mass Spectrometry to Rapidly Map Metabolic Flux of Substrates Labeled with Stable Isotopes

Metabolites
Direct infusion–high-resolution mass spectrometry (DI-HRMS) allows for rapid profiling of complex mixtures of metabolites in blood, cerebrospinal fluid, tissue samples and cultured cells.
Nils W. F. Meijer, Susan Zwakenberg, Johan Gerrits, Denise Westland, Arif I. Ardisasmita, Sabine A. Fuchs, Nanda M. Verhoeven-Duif, Judith J. M. Jans, Fried J. T. Zwartkruis   +8 more
doaj   +1 more source

Characterization and use of methylmalonyl-CoA mutase activity in Corynebacterium glutamicum

, 2009
Corynebacterium glutamicum has a very high capacity for the production of metabolites derived from central metabolism in which the citric acid cycle occupies a key position for the delivery of precursors for biosynthetic purposes.
LINDLEY, Nicholas David, BOTELLA, Laure
core  

Clinical Course of Methylmalonic Aciduria in Siblings: Two Clinical Cases

Вопросы современной педиатрии
Background. Methylmalonic aciduria (MMA) is a rare disease from the group of hereditary metabolic diseases. The MMA clinical picture is polymorphic and meanwhile similar to other metabolic disorders.
Olga V. Bugun, Galina P. Bogonosova, Tatyana A. Astakhova, Tatyana A. Bairova, Natalya N. Martynovich, Darya M. Barycova, Tatyana Y. Belkova, Yuliya S. Livadarova, Nanalya I. Sysoeva   +8 more
doaj   +1 more source

Caractérisation et utilisation de l'activité méthylmalonyl-CoA mutase chez Corynebacterium glutamicum [PDF]

, 2009
Corynebacterium glutamicum has a very high capacity for the production of metabolites derived from central metabolism in which the citric acid cycle occupies a key position for the delivery of precursors for biosynthetic purposes.
Botella, Laure
core  

Scheme of C₁ metabolism of the methylotroph Methylobacterium extorquens AM1.

, 2013
The bacterium oxidizes methanol to formaldehyde that is condensed with a tetrahydromethanopterin and further oxidized to formate. Formate reacts with tetrahydropterin and formyl-tetrahydrofolate is further converted to methylenetetrahydrofolate (part 1 ...
Georg Fuchs (361294), Hana Šmejkalová (361292), Tobias J. Erb (361293)   +2 more
core   +1 more source

Maternal Vitamin B12 in Pregnancy and Placental Development

Journal of Nutrition and Metabolism
Vitamin B12, or cobalamin, is an essential nutrient required for diverse physiological functions secondary to its role as a critical cofactor for two mammalian enzymes, methionine synthase and methylmalonyl-CoA mutase. While essential throughout all life
Amrita Arcot, Rachel E. Walker, Kelly Gallagher, Kevin C. Klatt, Alison D. Gernand   +4 more
doaj   +1 more source