Results 61 to 70 of about 2,001,857 (129)

Successful Treatment of Molybdenum Cofactor Deficiency Type A With cPMP

, 2010
Molybdenum cofactor deficiency (MoCD) is a rare metabolic disorder characterized by severe and rapidly progressive neurologic damage caused by the functional loss of sulfite oxidase, 1 of 4 molybdenum-dependent enzymes.
Jon Fairweather, Jochen Reiss, Schwarz, Günter, Joy Yaplito-lee, Veldman, Alex, Cook, Iain, Yaplito-Lee, Joy, Guenter Schwarz, Fairweather, Jon, Clive Andrew Ramsden, Alex Veldman, José Angel Santamaria-araujo, James Pitt, Sollazzo, Silvio, Silvio Sollazzo, Ramsden, Clive Andrew, Flora Wong, Schwarz, Guenter, Pitt, James, Santamaria-Araujo, Jose Angel, Gianello, Robert, Reiss, Jochen, Wong, Flora   +22 more
core   +1 more source

Sulfite determination using sulfite oxidase biosensor based glassy carbon electrode coated with thin mercury film

, 2007
The preparation of sulfite biosensor by immobilization of sulfite oxidase on a glassy carbon electrode coated with mercury thin film is described. Dissolved oxygen can be readily reduced at the glassy carbon electrode coated with mercury thin film.
Ertas, F. Nil, Akyilmaz, Erol, Sezginturk, Mustafa Kemal, Dinckaya, Erhan   +3 more
core   +1 more source

Isolated sulfite oxidase deficiency: a founder mutation. [PDF]

Cold Spring Harb Mol Case Stud, 2020
Mhanni AA, Greenberg CR, Spriggs EL, Agatep R, Sisk RR, Prasad C.   +5 more
europepmc   +1 more source

Effect of sulfite on antioxidant enzymes and lipid peroxidation in normal and sulfite oxidase-deficient rat erythrocytes

, 2010
Sulfite and related chemical such as sulfite salts and sulfur dioxide has been used as a preservative in food and drugs. This molecule has also been generated from the catabolism of sulfur-containing amino acids.
Suleyman Oktar, Vural Kucukatay, Ozturk, O.H., Küçükatay, Vural, Oktar, S., Oktay Hasan Ozturk, Aydin, M., Mehmet Aydin   +7 more
core   +1 more source

Expressions of N-methyl-d-aspartate receptors NR2A and NR2B subunit proteins in normal and sulfite-oxidase deficient rat’s hippocampus: effect of exogenous sulfite ingestion

, 2006
Sulfites whether ingested or produced through the sulfur-containing amino acids metabolism of the animal are very active molecules and can cause cellular toxicity.
Namik Delibaş, Öztürk, O.H., Hüseyin Bağci, Yönden, Z., Delibaş, N., Küçükatay, Vural, Aysel Ağar, Zafer Yönden, Ağar, A., Oktay Hasan Öztürk, Vural Küçükatay, Bağci, H., Delibas, Namik, Bagci, Huseyin, Ozturk, Oktay Hasan, Yonden, Zafer, Agar, Aysel   +16 more
core   +1 more source

Effect of sulfite on red blood cell deformability ex vivo and in normal and sulfite oxidase-deficient rats in vivo

, 2005
The effect of sulfite, a widely used food additive, on red blood cell deformability ex vivo and in vivo was investigated. Ex vivo experiments were conducted in human blood exposed to sulfite (5, 10 and 20 mM).
Melek Bor-Kucukatay, Vural Kucukatay, Baskurt, O.K., Küçükatay, Vural, Ağar, Aysel, Oguz Kerim Baskurt, Bor-Küçükatay, Melek, Aysel Agar   +7 more
core   +1 more source

Maternal uniparental isodisomy is responsible for serious molybdenum cofactor deficiency

, 2010
Molybdenum cofactor (MoCo) deficiency is a rare autosomal recessive inherited metabolic disorder resulting in the combined deficiency of aldehyde oxidase, xanthine dehydrogenase, and sulfite oxidase.
Gumus, Hakan, GAMZE POYRAZOĞLU, Poyrazoglu, Gamze, HAKAN GÜMÜŞ, Engelen, John, Dundar, Munis, Caglayan, Ahmet Okay, STIJN GHESQUIERE, AHMET OKAY ÇAĞLAYAN, MUNIS DUNDAR, Ichida, Kimiyoshi, Kumandas, Sefer, KIMIYOSHI ICHIDA, HÜSEYIN PER, Ghesquiere, Stijn, Kondolot, Meda, MEDA KONDOLOT, Per, HÜSEYİN, SEFER KUMANDAŞ, JOHN ENGELEN, Per, Hueseyin   +20 more
core   +1 more source

Prenatal Brain Disruption in Molybdenum Cofactor Deficiency

, 2011
Molybdenum cofactor deficiency is a rare autosomal recessive disorder that may present during the neonatal period with intractable seizures and be mistaken for ischemic encephalopathy.
Hanna Mandel, Nirit Carmi-Nawi, Gustavo Malinger, Dorit Lev, Kimiyoshi Ichida, Tally Lerman-Sagie   +5 more
core   +1 more source

Spectroscopic Characterization of YedY: The Role of Sulfur Coordination in a Mo(V) Sulfite Oxidase Family Enzyme Form

, 2016
Spectroscopic Characterization of YedY: The Role of Sulfur Coordination in a Mo(V) Sulfite Oxidase Family Enzyme ...
Martin L. Kirk (1327839), Joel H. Weiner (180389), Jing Yang (13969), Joseph Sempombe (2179906), Richard Rothery (2340535)   +4 more
core   +1 more source

Effect of sulfite exposure on zinc, iron, and copper levels in rat liver and kidney tissues

, 2006
Sulfite is a potentially toxic molecule that might enter the body via ingestion, inhalation, or injection. For cellular detoxification, mammalians rely on sulfite oxidase to convert sulfite to sulfate.
Akça, Hakan, Turgut, S., Bor-Kucukatay, M., Emmungil, G., Küçükatay, Vural, Turgut, G., Kocamaz, Erdoğan   +6 more
core   +1 more source