Zu "K00253" wurden 12 Artikel gefunden!
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Isovaleroylglycine
Artikelnummer: Cay17581-500
Isovaleroylglycine is a compound found in the urine of patients with isovaleric acidemia, a disorder created by defective leucine metabolism due to a deficiency in the isovaleryl coenzyme A dehydrogenase. It has been used as a diagnostic tool in the diagnosis of various acidemias and mitochondrial fatty acid...
| Schlagworte: | N-Isovalerylglycine, N-(3-methyl-1-oxobutyl)-glycine |
| CAS | 16284-60-9 |
| MW: | 159.2 D |
ab 60,00 €
Anti-IVD
Artikelnummer: E-AB-65247.120
Isovaleryl-CoA dehydrogenase (IVD) is a mitochondrial matrix enzyme that catalyzes the third step in leucine catabolism. The genetic deficiency of IVD results in an accumulation of isovaleric acid, which is toxic to the central nervous system and leads to isovaleric acidemia. Alternatively spliced transcript...
| Schlagworte: | Anti-IVD, EC=1.3.8.4, Anti-Isovaleryl-CoA dehydrogenase, mitochondrial, IVD Polyclonal Antibody |
| Anwendung: | WB |
| Wirt: | Rabbit |
| Spezies-Reaktivität: | human, mouse |
ab 198,00 €
Isovaleryl-DL-carnitine-d3 (chloride)
Artikelnummer: Cay26573-1
Isovaleryl-DL-carnitine-d3 is intended for use as an internal standard for the quantification of isovaleryl-DL-carnitine by GC- or LC-MS. Isovaleryl-DL-carnitine is a derivative of carnitine (Cay-21489, Cay-16749). Increased levels of isovaleryl carnitine are associated with isovaleric acidemia, an inborn error of...
| Schlagworte: | DL-Isovalerylcarnitine-d3, 3-carboxy-N,N-dimethyl-N-(methyl-d3)-2-((3-methylbutanoyl)oxy)propan-1-aminium, monochloride |
| Anwendung: | GC-MS, LC-MS, quantification, internal standard, isovaleryl-CoA dehydrogenase defiency marker |
| MW: | 278.7 D |
ab 92,00 €
Anti-IVD
Artikelnummer: ARG58891.100
| Schlagworte: | Anti-IVD, EC=1.3.8.4, Anti-Isovaleryl-CoA dehydrogenase, mitochondrial |
| Anwendung: | WB |
| Wirt: | Rabbit |
| Spezies-Reaktivität: | human, mouse |
624,00 €
Anti-IVD
Artikelnummer: E-AB-18718.120
IVD(Isovaleryl CoA dehydrogenase,mitochondrial) is a member of the acylCoA dehydrogenase family and is involved in the catabolism of leucine. In size,IVD precursor and mature proteins produced by class I mutants are indistinguishable from their normal counterparts. Class II,III,and IV mutants make IVD precursor...
| Schlagworte: | Anti-IVD, EC=1.3.8.4, Anti-Isovaleryl-CoA dehydrogenase, mitochondrial, IVD Polyclonal Antibody |
| Anwendung: | WB, ELISA |
| Wirt: | Rabbit |
| Spezies-Reaktivität: | human, mouse, rat |
ab 71,00 €
Anti-IVD
Artikelnummer: ATA-HPA041391.100
Protein function: Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl- CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway (PubMed:7640268). To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as...
| Schlagworte: | Anti-IVD, Anti-Butyryl-CoA dehydrogenase, Anti-Isovaleryl-CoA dehydrogenase, mitochondrial |
| Anwendung: | ICC, IHC, WB |
| Wirt: | Rabbit |
| Spezies-Reaktivität: | human |
446,00 €
Isovaleryl-DL-carnitine-d9 (chloride)
Artikelnummer: Cay37213-1
Isovaleryl-DL-carnitine-d9 is intended for use as an internal standard for the quantification of isovaleryl-DL-carnitine by GC- or LC-MS. Isovaleryl-DL-carnitine is a derivative of carnitine (Cay-21489, Cay-16749). Increased levels of isovaleryl carnitine are associated with isovaleric acidemia, an inborn error of...
| Schlagworte: | DL-Isovalerylcarnitine-d9, 3-carboxy-N,N,N-tris(methyl-d3)-2-((3-methylbutanoyl)oxy)propan-1-aminium, monochloride |
| Anwendung: | GC-MS, LC-MS internal standard, quantification, isovaleryl-CoA dehydrogenase defiency marker |
| CAS | 2608872-54-2 |
| MW: | 290.8 D |
ab 147,00 €
Anti-IVD
Artikelnummer: ELK-ES19827.100
Protein function: Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl- CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway (PubMed:7640268). To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as...
| Schlagworte: | Anti-IVD, Anti-Butyryl-CoA dehydrogenase, Anti-Isovaleryl-CoA dehydrogenase, mitochondrial, IVD rabbit pAb |
| Anwendung: | WB |
| Wirt: | Rabbit |
| Spezies-Reaktivität: | human, mouse, rat |
ab 169,00 €
IVD, Recombinant, Human, aa33-426, His-Tag...
Artikelnummer: 350013.100
IVD (Isovaleryl Coenzyme A dehydrogenase) is a mitochondrial matrix enzyme that belongs to the acyl-CoA dehydrogenase family. IVD is a homotetrameric flavoenzyme which catalyzes the conversion of isovaleryl-CoA to 3-methylcrotonyl-CoA. Defects of the IVD gene lead to ineffective isoforms that are the underlying...
| Schlagworte: | IVD, Butyryl-CoA dehydrogenase, Isovaleryl-CoA dehydrogenase, mitochondrial |
| Ursprungsart: | human |
| MW: | 45.3 kD |
ab 537,00 €
Human IVD (Isovaleryl-CoA dehydrogenase) ELISA Kit...
Artikelnummer: G-HUFI03782.96
Human IVD (Isovaleryl-CoA dehydrogenase) ELISA Kit from Assay Genie is a pre-coated immunoassay with a high sensitivity and broad range and has been designed to measure Human IVD (Isovaleryl-CoA dehydrogenase) in serum, plasma & cell culture supernatant samples. The Human IVD (Isovaleryl-CoA dehydrogenase) ELISA Kit...
| Schlagworte: | IVD, Butyryl-CoA dehydrogenase, Isovaleryl-CoA dehydrogenase, mitochondrial |
| Anwendung: | Sandwich ELISA |
| Spezies-Reaktivität: | human |
641,00 €
IVD PrEST Antigen
Artikelnummer: ATA-APrEST81459.100
Protein function: Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl- CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway (PubMed:7640268). To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as...
| Schlagworte: | IVD, Butyryl-CoA dehydrogenase, Isovaleryl-CoA dehydrogenase, mitochondrial |
| Anwendung: | Control antigen |
| Exprimiert in: | E.coli |
| Ursprungsart: | human |
247,00 €
Anti-IVD
Artikelnummer: G-CAB15281.20
Protein function: Catalyzes the conversion of isovaleryl-CoA/3-methylbutanoyl- CoA to 3-methylbut-2-enoyl-CoA as an intermediate step in the leucine (Leu) catabolic pathway (PubMed:7640268). To a lesser extent, is also able to catalyze the oxidation of other saturated short-chain acyl-CoA thioesters as...
| Schlagworte: | Anti-IVD, Anti-Butyryl-CoA dehydrogenase, Anti-Isovaleryl-CoA dehydrogenase, mitochondrial |
| Anwendung: | WB, IHC |
| Wirt: | Rabbit |
| Spezies-Reaktivität: | human, mouse, rat |
149,00 €