You Searched For: Beckman Coulter

Catalog Number: (BOSSBS-13312R-A555)

Supplier: Bioss

Description: 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

Sale

Catalog Number: (BOSSBS-13312R-A350)

Supplier: Bioss

Description: 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

Sale

Catalog Number: (BOSSBS-0494R-CY5.5)

Supplier: Bioss

Description: 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

Sale

Catalog Number: (MOLE30858763-500G)

Supplier: Molekula

Description: Glutaric acid

UOM: 1 * 500 g

Sale

 This is a MarketSource item. Additional charges may apply

Catalog Number: (SIALD96006-100G)

Supplier: Merck

Description: Diethyl glutarate, Sigma-Aldrich®

UOM: 1 * 100 g

Sale

Supplier: Merck

Description: Glutaric acid, Sigma-Aldrich®

Certificates

Catalog Number: (MOLEM11019138)

Supplier: Molekula

Description: Glutaric acid

UOM: 1 * 100 g

Sale

 This is a MarketSource item. Additional charges may apply

Catalog Number: (EHERC14034500)

Supplier: EHRENSTORFER

Description: Glutaric acid

UOM: 1 * 0,25 g

Sale

Catalog Number: (BOSSBS-13312R-CY5)

Supplier: Bioss

Description: 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

Sale

Catalog Number: (BOSSBS-13312R-A680)

Supplier: Bioss

Description: 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

Sale

Catalog Number: (SPCMD2948-200LTBL)

Supplier: Spectrum Chemical

Description: Dimethyl Glutarate is used in de-icing and anti-icing applications, construction and road applications, and release and binders agents.

UOM: 1 * 200 L

Sale

Supplier: Merck

Description: Methyl hydrogen glutarate, Sigma-Aldrich®

Catalog Number: (BOSSBS-0494R-HRP)

Supplier: Bioss

Description: 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

Sale

Catalog Number: (BOSSBS-0494R-A750)

Supplier: Bioss

Description: 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

Sale

Catalog Number: (BOSSBS-0494R-CY5)

Supplier: Bioss

Description: 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

Sale

Supplier: Thermo Fisher Scientific

Description: Thermo Scientific Pierce DSG is a water-insoluble, homo-bifunctional N-hydroxysuccinimide ester (NHS-ester) crosslinker often used for conjugating radiolabeled ligands to cell-surface receptors. The NHS ester is the simplest and most commonly used reactive group for crosslinking and labeling proteins and peptides. NHS esters react with primary amines on the N-termini of peptides and the  amine of lysine residues, forming a stable, covalent amide bond and releasing the NHS group.