Hai cercato: DSG+(Disuccinimidyl+glutarate)

Codice catalogo: (BOSSBS-0494R-A488)

Fornitore: Bioss

Codice articolo fornitore: BS-0494R-A488

Codice articolo locale: BOSSBS-0494R-A488

Descrizione: ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008].

UOM: 1 * 100 µl

Promo

Codice catalogo: (BOSSBS-0494R-CY7)

Fornitore: Bioss

Codice articolo fornitore: BS-0494R-CY7

Codice articolo locale: BOSSBS-0494R-CY7

Descrizione: ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008].

UOM: 1 * 100 µl

Promo

Codice catalogo: (BOSSBS-0494R-A555)

Fornitore: Bioss

Codice articolo fornitore: BS-0494R-A555

Codice articolo locale: BOSSBS-0494R-A555

Descrizione: ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008].

UOM: 1 * 100 µl

Promo

Codice catalogo: (BOSSBS-1294R-CY7)

Fornitore: Bioss

Codice articolo fornitore: BS-1294R-CY7

Codice articolo locale: BOSSBS-1294R-CY7

Descrizione: Transports C5-C7 oxodicarboxylates across the inner membranes of mitochondria. Can transport 2-oxoadipate, 2-oxoglutarate, adipate, glutarate, and to a lesser extent, pimelate, 2-oxopimelate, 2-aminoadipate, oxaloacetate, and citrate.

UOM: 1 * 100 µl

Promo

Codice catalogo: (BOSSBS-13312R-A647)

Fornitore: Bioss

Codice articolo fornitore: BS-13312R-A647

Codice articolo locale: BOSSBS-13312R-A647

Descrizione: GCDH is a 438 amino acid protein that localizes to the mitochondrial matrix and belongs to the acyl-CoA dehydrogenase family. Existing as a homotetramer, GCDH uses FAD as a cofactor to catalyze the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine and L-tryptophan metabolism. While GCDH exists as both a long and short isoform, only the long isoform is a functionally active protein. Defects in the gene encoding GCDH are the cause of glutaric acidemia type I (GA-I), an autosomal recessive disorder that is characterized by the accumulation of glutaconic acid and is associated with such symptoms as progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.

UOM: 1 * 100 µl

Promo

Codice catalogo: (BOSSBS-13312R-CY3)

Fornitore: Bioss

Codice articolo fornitore: BS-13312R-CY3

Codice articolo locale: BOSSBS-13312R-CY3

Descrizione: GCDH is a 438 amino acid protein that localizes to the mitochondrial matrix and belongs to the acyl-CoA dehydrogenase family. Existing as a homotetramer, GCDH uses FAD as a cofactor to catalyze the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine and L-tryptophan metabolism. While GCDH exists as both a long and short isoform, only the long isoform is a functionally active protein. Defects in the gene encoding GCDH are the cause of glutaric acidemia type I (GA-I), an autosomal recessive disorder that is characterized by the accumulation of glutaconic acid and is associated with such symptoms as progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.

UOM: 1 * 100 µl

Promo

Codice catalogo: (BOSSBS-0494R-A680)

Fornitore: Bioss

Codice articolo fornitore: BS-0494R-A680

Codice articolo locale: BOSSBS-0494R-A680

Descrizione: ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene.

UOM: 1 * 100 µl

Promo

Codice catalogo: (BOSSBS-0494R-FITC)

Fornitore: Bioss

Codice articolo fornitore: BS-0494R-FITC

Codice articolo locale: BOSSBS-0494R-FITC

Descrizione: ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008].

UOM: 1 * 100 µl

Promo

Codice catalogo: (BOSSBS-1294R-HRP)

Fornitore: Bioss

Codice articolo fornitore: BS-1294R-HRP

Codice articolo locale: BOSSBS-1294R-HRP

Descrizione: Transports C5-C7 oxodicarboxylates across the inner membranes of mitochondria. Can transport 2-oxoadipate, 2-oxoglutarate, adipate, glutarate, and to a lesser extent, pimelate, 2-oxopimelate, 2-aminoadipate, oxaloacetate, and citrate.

UOM: 1 * 100 µl

Promo

Codice catalogo: (BOSSBS-13312R-A750)

Fornitore: Bioss

Codice articolo fornitore: BS-13312R-A750

Codice articolo locale: BOSSBS-13312R-A750

Descrizione: GCDH is a 438 amino acid protein that localizes to the mitochondrial matrix and belongs to the acyl-CoA dehydrogenase family. Existing as a homotetramer, GCDH uses FAD as a cofactor to catalyze the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine and L-tryptophan metabolism. While GCDH exists as both a long and short isoform, only the long isoform is a functionally active protein. Defects in the gene encoding GCDH are the cause of glutaric acidemia type I (GA-I), an autosomal recessive disorder that is characterized by the accumulation of glutaconic acid and is associated with such symptoms as progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.

UOM: 1 * 100 µl

Promo

Codice catalogo: (BOSSBS-13312R-FITC)

Fornitore: Bioss

Codice articolo fornitore: BS-13312R-FITC

Codice articolo locale: BOSSBS-13312R-FITC

Descrizione: GCDH is a 438 amino acid protein that localizes to the mitochondrial matrix and belongs to the acyl-CoA dehydrogenase family. Existing as a homotetramer, GCDH uses FAD as a cofactor to catalyze the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine and L-tryptophan metabolism. While GCDH exists as both a long and short isoform, only the long isoform is a functionally active protein. Defects in the gene encoding GCDH are the cause of glutaric acidemia type I (GA-I), an autosomal recessive disorder that is characterized by the accumulation of glutaconic acid and is associated with such symptoms as progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.

UOM: 1 * 100 µl

Promo

Codice catalogo: (BOSSBS-1294R-A488)

Fornitore: Bioss

Codice articolo fornitore: BS-1294R-A488

Codice articolo locale: BOSSBS-1294R-A488

Descrizione: Transports C5-C7 oxodicarboxylates across the inner membranes of mitochondria. Can transport 2-oxoadipate, 2-oxoglutarate, adipate, glutarate, and to a lesser extent, pimelate, 2-oxopimelate, 2-aminoadipate, oxaloacetate, and citrate.

UOM: 1 * 100 µl

Promo

Codice catalogo: (BOSSBS-0494R-A647)

Fornitore: Bioss

Codice articolo fornitore: BS-0494R-A647

Codice articolo locale: BOSSBS-0494R-A647

Descrizione: ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008].

UOM: 1 * 100 µl

Promo

Codice catalogo: (EHERC14034900)

Fornitore: EHRENSTORFER

Codice articolo fornitore: C14034900

Codice articolo locale: EHERC14034900

Descrizione: Dimethyl glutarate

UOM: 1 * 0,25 g

Promo

Codice catalogo: (BOSSBS-13312R-A488)

Fornitore: Bioss

Codice articolo fornitore: BS-13312R-A488

Codice articolo locale: BOSSBS-13312R-A488

Descrizione: GCDH is a 438 amino acid protein that localizes to the mitochondrial matrix and belongs to the acyl-CoA dehydrogenase family. Existing as a homotetramer, GCDH uses FAD as a cofactor to catalyze the oxidative decarboxylation of glutaryl-CoA to crotonyl-CoA and CO(2) in the degradative pathway of L-lysine, L-hydroxylysine and L-tryptophan metabolism. While GCDH exists as both a long and short isoform, only the long isoform is a functionally active protein. Defects in the gene encoding GCDH are the cause of glutaric acidemia type I (GA-I), an autosomal recessive disorder that is characterized by the accumulation of glutaconic acid and is associated with such symptoms as progressive dystonia and athetosis due to gliosis and neuronal loss in the basal ganglia.

UOM: 1 * 100 µl

Promo

Codice catalogo: (BOSSBS-0494R-CY5.5)

Fornitore: Bioss

Codice articolo fornitore: BS-0494R-CY5.5

Codice articolo locale: BOSSBS-0494R-CY5.5

Descrizione: ETFA participates in catalyzing the initial step of the mitochondrial fatty acid beta-oxidation. It shuttles electrons between primary flavoprotein dehydrogenases and the membrane-bound electron transfer flavoprotein ubiquinone oxidoreductase. Defects in electron-transfer-flavoprotein have been implicated in type II glutaricaciduria in which multiple acyl-CoA dehydrogenase deficiencies result in large excretion of glutaric, lactic, ethylmalonic, butyric, isobutyric, 2-methyl-butyric, and isovaleric acids. Two transcript variants encoding different isoforms have been found for this gene. [provided by RefSeq, Jul 2008].

UOM: 1 * 100 µl

Promo