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Methylmalonic acid 96%

Supplier: Thermo Fisher Scientific
Description: Methylmalonic acid 96%

MSDS

Image Unavailable-67649-10MG

Supelco® Organic Reference Standard, Methylmalonic Acid

Supplier: Merck
Description: Organic Standard, Methylmalonic acid

Image Unavailable-M-080-1ML

Supelco® Cerilliant® Organic Reference Standard, Methylmalonic acid solution

Catalog Number: (M-080-1ML)
Supplier: Merck
Description: Organic Standard, Methylmalonic acid solution, Ampule of 1 ml
UOM: 1 * 1 mL
Image Unavailable-APOSOR911347-100G

Methylmalonic acid 97%

Catalog Number: (APOSOR911347-100G)
Supplier: Apollo Scientific
Description: Methylmalonic acid 97%
UOM: 1 * 100 g
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Diethyl-2-bromo-2-methylmalonate 98%

Supplier: Thermo Fisher Scientific
Description: Diethyl-2-bromo-2-methylmalonate 98%

MSDS Certificates

Product Image-ACRO155145000

Diethyl methylmalonate 99%

Supplier: Thermo Fisher Scientific
Description: Diethyl methylmalonate 99%

MSDS

Image Unavailable-BOSSBS-9808R-A680

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

Catalog Number: (BOSSBS-9808R-A680)
Supplier: Bioss
Description: 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)

Catalog Number: (BOSSBS-9808R-A750)
Supplier: Bioss
Description: 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-CY7

Anti-HIBADH Rabbit Polyclonal Antibody (Cy7)

Catalog Number: (BOSSBS-15483R-CY7)
Supplier: Bioss
Description: 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-CY3

Anti-HIBADH Rabbit Polyclonal Antibody (Cy3)

Catalog Number: (BOSSBS-15483R-CY3)
Supplier: Bioss
Description: 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-FITC

Anti-HIBADH Rabbit Polyclonal Antibody (FITC)

Catalog Number: (BOSSBS-15483R-FITC)
Supplier: Bioss
Description: 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-CY3

Anti-ENPP6 Rabbit Polyclonal Antibody (Cy3®)

Catalog Number: (BOSSBS-13076R-CY3)
Supplier: Bioss
Description: 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-15483R

Anti-HIBADH Rabbit Polyclonal Antibody

Catalog Number: (BOSSBS-15483R)
Supplier: Bioss
Description: HIBADH is a 336 amino acid mitochondrial enzyme that catalyzes the NAD+-dependent, reversible oxidization 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 hypothesized that defects in the gene encoding HIBADH may be the cause of 3-Hydroxyisobutyric aciduria, a rare disorder that is characterized 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-A647

Anti-HIBADH Rabbit Polyclonal Antibody (ALEXA FLUOR® 647)

Catalog Number: (BOSSBS-15483R-A647)
Supplier: Bioss
Description: 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-9991R-A680

Anti-C4orf22 Rabbit Polyclonal Antibody (Alexa Fluor® 680)

Catalog Number: (BOSSBS-9991R-A680)
Supplier: Bioss
Description: C4orf22 is a 233 amino acid protein that exists as three alternatively spliced isoforms and are encoded by a gene encoding maping to human chromosome 4, which 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 maps to 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
Image Unavailable-BOSSBS-9995R-A680

Anti-C4orf32 Rabbit Polyclonal Antibody (Alexa Fluor® 680)

Catalog Number: (BOSSBS-9995R-A680)
Supplier: Bioss
Description: C4orf32 is a 132 amino acid single pass membrane protein that is 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 located on chromosome 4. FGFR-3 is also encoded by a gene that maps to human 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
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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 on +353 1 88 22222.
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 on +353 1 88 22222.
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 eurega_services@eu.vwr.com
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The original product is no longer available. The replacement shown is available.
Product(s) marked with this symbol are discontinued - sold till end of stock. Alternatives may be available by searching with the VWR Catalog Number listed above. If you need further assistance, please call VWR Customer Service on +353 1 8822222.
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