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Codice catalogo: (M-080-1ML)
Fornitore: Merck
Codice articolo fornitore: M-080-1ML
Codice articolo locale: SUPLM-080-1ML
Descrizione: Organic Standard, Methylmalonic acid solution, Ampule of 1 ml
UOM: 1 * 1 mL


Fornitore: Merck
Descrizione: Organic Standard, Methylmalonic acid

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Fornitore: TCI EUROPE
Descrizione: Methylmalonic acid ≥98.0% (by titrimetric analysis)

Fornitore: TCI EUROPE
Descrizione: Diethyl-2-ethyl-2-methylmalonate ≥97.0% (by GC)

Fornitore: Thermo Scientific
Descrizione: Methylmalonic acid 96%
Fornitore: Apollo Scientific
Descrizione: Methylmalonic acid 97%

Fornitore: Thermo Scientific
Descrizione: Diethyl-2-bromo-2-methylmalonate 98%
Codice catalogo: (TCIAD4368-25G)
Fornitore: TCI EUROPE
Codice articolo fornitore: D4368-25G
Codice articolo locale: TCIAD4368-25G
Descrizione: Diethyl-2-bromo-2-methylmalonate ≥98.0% (by GC, titration analysis)
UOM: 1 * 25 g


Codice catalogo: (BOSSBS-15483R-CY3)
Fornitore: Bioss
Codice articolo fornitore: BS-15483R-CY3
Codice articolo locale: BOSSBS-15483R-CY3
Descrizione: HIBADH is a 336 amino acid mitochondrial enzyme that catalyses the NAD+-dependent, reversible oxidisation of 3-Hydroxyisobutyrate to methylmalonate semialdehyde, an intermediate of valine catabolism. The enzyme functions as a homodimer between a pH of 7,0 and 10,0, with optimal activity between 8,8 and 9,0. It was previously hypothesised that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterised by a variety of clinical manifestations such as neurodevelopmental problems and dysmorphic features. However, it was shown that HIBADH activity was equal in patients with 3-Hydroxyisobutyric aciduria as compared with controls.
UOM: 1 * 100 µl


Codice catalogo: (BOSSBS-9808R-A680)
Fornitore: Bioss
Codice articolo fornitore: BS-9808R-A680
Codice articolo locale: BOSSBS-9808R-A680
Descrizione: C2ORF25 is a mitochondrial protein and its function is not fully identified. But, Vitamin B12 (cobalamin) is an essential cofactor in several metabolic pathways. Intracellular conversion of cobalamin to adenosylcobalamin in mitochondria and to methylcobalamin in cytoplasm is necessary for homeostasis of methylmalonic acid and homocysteine. C2ORF25 encodes a protein involved in an early step of cobalamin metabolism.
UOM: 1 * 100 µl


Codice catalogo: (BOSSBS-15187R)
Fornitore: Bioss
Codice articolo fornitore: BS-15187R
Codice articolo locale: BOSSBS-15187R
Descrizione: C4orf17 (chromosome 4 open reading frame 17) is a 359 amino acid protein encoded by a gene that maps to human chromosome 4. Human chromosome 4 represents approximately 6% of the human genome and contains nearly 900 genes. Notably, the Huntingtin gene, which is found to encode an expanded glutamine tract in cases of Huntington's disease, is on chromosome 4. FGFR-3 is also encoded by a gene that is located on chromosome 4, and has been associated with thanatophoric dwarfism, achondroplasia, Muenke syndrome and bladder cancer. Chromosome 4 is also tied to Ellis-van Creveld syndrome, methylmalonic acidemia and polycystic kidney disease.
UOM: 1 * 100 µl


Fornitore: TCI EUROPE
Descrizione: Diethyl methylmalonate ≥98.0% (by GC)

Codice catalogo: (BOSSBS-13076R-HRP)
Fornitore: Bioss
Codice articolo fornitore: BS-13076R-HRP
Codice articolo locale: BOSSBS-13076R-HRP
Descrizione: NPP6 is a 440 amino acid member of the nucleotide pyrophosphatase/phosphodiesterase family. NPP6 is a secreted and single-pass type I membrane protein. Predominantly expressed in brain and kidney, NPP6 is a choline-specific glycerophosphodiester phosphodiesterase. NPP6 can hydrolyze the classical substrate for phospholipase C, p-nitrophenyl phosphorylcholine, glycerophosphorylcholine, sphingosylphosphorylcholine and lysophosphatidylcholine (LPC). NPP6 has been found to have a preference for LPC with polyunsaturated or short fatty acids. The gene encoding NPP6 maps to human chromosome 4, which consists of approximately 6% of the human genome and nearly 900 genes. Chromosome 4 is associated with Huntington's disease, FGFR-3, Ellis-van Creveld syndrome, methylmalonic acidemia and polycystic kidney disease.
UOM: 1 * 100 µl


Codice catalogo: (BOSSBS-13076R-A680)
Fornitore: Bioss
Codice articolo fornitore: BS-13076R-A680
Codice articolo locale: BOSSBS-13076R-A680
Descrizione: NPP6 is a 440 amino acid member of the nucleotide pyrophosphatase/phosphodiesterase family. NPP6 is a secreted and single-pass type I membrane protein. Predominantly expressed in brain and kidney, NPP6 is a choline-specific glycerophosphodiester phosphodiesterase. NPP6 can hydrolyze the classical substrate for phospholipase C, p-nitrophenyl phosphorylcholine, glycerophosphorylcholine, sphingosylphosphorylcholine and lysophosphatidylcholine (LPC). NPP6 has been found to have a preference for LPC with polyunsaturated or short fatty acids. The gene encoding NPP6 maps to human chromosome 4, which consists of approximately 6% of the human genome and nearly 900 genes. Chromosome 4 is associated with Huntington's disease, FGFR-3, Ellis-van Creveld syndrome, methylmalonic acidemia and polycystic kidney disease.
UOM: 1 * 100 µl


Codice catalogo: (BOSSBS-13076R-A488)
Fornitore: Bioss
Codice articolo fornitore: BS-13076R-A488
Codice articolo locale: BOSSBS-13076R-A488
Descrizione: NPP6 is a 440 amino acid member of the nucleotide pyrophosphatase/phosphodiesterase family. NPP6 is a secreted and single-pass type I membrane protein. Predominantly expressed in brain and kidney, NPP6 is a choline-specific glycerophosphodiester phosphodiesterase. NPP6 can hydrolyze the classical substrate for phospholipase C, p-nitrophenyl phosphorylcholine, glycerophosphorylcholine, sphingosylphosphorylcholine and lysophosphatidylcholine (LPC). NPP6 has been found to have a preference for LPC with polyunsaturated or short fatty acids. The gene encoding NPP6 maps to human chromosome 4, which consists of approximately 6% of the human genome and nearly 900 genes. Chromosome 4 is associated with Huntington's disease, FGFR-3, Ellis-van Creveld syndrome, methylmalonic acidemia and polycystic kidney disease.
UOM: 1 * 100 µl


Codice catalogo: (BOSSBS-13076R-A750)
Fornitore: Bioss
Codice articolo fornitore: BS-13076R-A750
Codice articolo locale: BOSSBS-13076R-A750
Descrizione: NPP6 is a 440 amino acid member of the nucleotide pyrophosphatase/phosphodiesterase family. NPP6 is a secreted and single-pass type I membrane protein. Predominantly expressed in brain and kidney, NPP6 is a choline-specific glycerophosphodiester phosphodiesterase. NPP6 can hydrolyze the classical substrate for phospholipase C, p-nitrophenyl phosphorylcholine, glycerophosphorylcholine, sphingosylphosphorylcholine and lysophosphatidylcholine (LPC). NPP6 has been found to have a preference for LPC with polyunsaturated or short fatty acids. The gene encoding NPP6 maps to human chromosome 4, which consists of approximately 6% of the human genome and nearly 900 genes. Chromosome 4 is associated with Huntington's disease, FGFR-3, Ellis-van Creveld syndrome, methylmalonic acidemia and polycystic kidney disease.
UOM: 1 * 100 µl


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