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Image Unavailable-M-080-1ML

Supelco® Cerilliant® Organic Reference Standard, Methylmalonic acid solution

Catalogus nummer: (M-080-1ML)
Leverancier: Merck
Omschrijving: Organic Standard, Methylmalonic acid solution, Ampule of 1 ml
UOM: 1 * 1 mL
Image Unavailable-67649-10MG

Supelco® Organic Reference Standard, Methylmalonic Acid

Leverancier: Merck
Omschrijving: Organic Standard, Methylmalonic acid

Image Unavailable-APOSOR911347-100G

Methylmalonic acid 97%

Catalogus nummer: (APOSOR911347-100G)
Leverancier: Apollo Scientific
Omschrijving: Methylmalonic acid 97%
UOM: 1 * 100 g
Image Unavailable-TCIAM0568-25G

Methylmalonic acid ≥98.0% (by titrimetric analysis)

Leverancier: TCI
Omschrijving: Methylmalonic acid ≥98.0% (by titrimetric analysis)

Product Image-ACRO127090250

Methylmalonic acid 96%

Leverancier: Thermo Fisher Scientific
Omschrijving: Methylmalonic acid 96%

MSDS

Image Unavailable-TCIAD3630-25G

Diethyl-2-ethyl-2-methylmalonate ≥97.0% (by GC)

Leverancier: TCI
Omschrijving: Diethyl-2-ethyl-2-methylmalonate ≥97.0% (by GC)

Image Unavailable-L00575.22

Diethyl-2-bromo-2-methylmalonate 98%

Leverancier: Thermo Fisher Scientific
Omschrijving: Diethyl-2-bromo-2-methylmalonate 98%

MSDS Certificaten

Image Unavailable-TCIAD4368-25G

Diethyl-2-bromo-2-methylmalonate ≥98.0% (by GC, titration analysis)

Catalogus nummer: (TCIAD4368-25G)
Leverancier: TCI
Omschrijving: Diethyl-2-bromo-2-methylmalonate ≥98.0% (by GC, titration analysis)
UOM: 1 * 25 g
Image Unavailable-TCIAM0366-500ML

Diethyl methylmalonate ≥98.0% (by GC)

Leverancier: TCI
Omschrijving: Diethyl methylmalonate ≥98.0% (by GC)

Product Image-ACRO155145000

Diethyl methylmalonate 99%

Leverancier: Thermo Fisher Scientific
Omschrijving: Diethyl methylmalonate 99%

MSDS

Image Unavailable-BOSSBS-9808R-A680

Anti-C2ORF25 Rabbit Polyclonal Antibody (Alexa Fluor® 680)

Catalogus nummer: (BOSSBS-9808R-A680)
Leverancier: Bioss
Omschrijving: 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
Image Unavailable-BOSSBS-9808R-A750

Anti-C2ORF25 Rabbit Polyclonal Antibody (Alexa Fluor® 750)

Catalogus nummer: (BOSSBS-9808R-A750)
Leverancier: Bioss
Omschrijving: 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
Image Unavailable-BOSSBS-15483R-CY3

Anti-HIBADH Rabbit Polyclonal Antibody (Cy3)

Catalogus nummer: (BOSSBS-15483R-CY3)
Leverancier: Bioss
Omschrijving: 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
Image Unavailable-BOSSBS-15483R-HRP

Anti-HIBADH Rabbit Polyclonal Antibody (HRP)

Catalogus nummer: (BOSSBS-15483R-HRP)
Leverancier: Bioss
Omschrijving: 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
Image Unavailable-BOSSBS-13076R-HRP

Anti-ENPP6 Rabbit Polyclonal Antibody (HRP (Horseradish Peroxidase))

Catalogus nummer: (BOSSBS-13076R-HRP)
Leverancier: Bioss
Omschrijving: 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
Image Unavailable-BOSSBS-13076R-A680

Anti-ENPP6 Rabbit Polyclonal Antibody (Alexa Fluor® 680)

Catalogus nummer: (BOSSBS-13076R-A680)
Leverancier: Bioss
Omschrijving: 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
Bel voor prijs
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Stock for this item is limited, but may be available in a warehouse close to you. Please make sure that you are logged in to the site so that available stock can be displayed. If the call is still displayed and you need assistance, please call us at 1-800-932-5000.
This product is marked as restricted and can only be purchased by approved Shipping Accounts. If you need further assistance, email VWR Regulatory Department at Regulatory_Affairs@vwr.com
-Additional Documentation May be needed to purchase this item. A VWR representative will contact you if needed.
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