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Image Unavailable-BOSSBS-15484R-HRP

Anti-HIBCH Rabbit Polyclonal Antibody (HRP)

Catalog Number: (BOSSBS-15484R-HRP)
Supplier: Bioss
Description: HIBCH is a 386 amino acid protein belonging to the enoyl-CoA hydratase/isomerase family. Localising to the mitochondria, HIBCH is highly expressed in liver and kidney, with lower levels found in heart, muscle and brain. HIBCH hydrolyses HIBYL-CoA, a saline catabolite, and _-hydroxypropionyl-CoA, an intermediate in the minor pathway involved in the metabolism of proprionate. Existing as two alternatively spliced isoforms, the gene encoding HIBCH maps to human chromosome 2q32.2. Defects to this gene result in HIBCH deficiency (HIBCHD), known alternatively as deficiency of _-hydroxyisobutyryl CoA deacylase or methacrylic aciduria. HIBCHD is characterised by the accumulation of methacrylyl-CoA, a highly reactive compound that undergoes addition reactions with free sulfhydryl groups. Phenotypic symptoms include early deterioration of neurological function, delayed motor skill development and hypotonia.
UOM: 1 * 100 µl
Image Unavailable-BOSSBS-15484R-CY3

Anti-HIBCH Rabbit Polyclonal Antibody (Cy3)

Catalog Number: (BOSSBS-15484R-CY3)
Supplier: Bioss
Description: HIBCH is a 386 amino acid protein belonging to the enoyl-CoA hydratase/isomerase family. Localising to the mitochondria, HIBCH is highly expressed in liver and kidney, with lower levels found in heart, muscle and brain. HIBCH hydrolyses HIBYL-CoA, a saline catabolite, and _-hydroxypropionyl-CoA, an intermediate in the minor pathway involved in the metabolism of proprionate. Existing as two alternatively spliced isoforms, the gene encoding HIBCH maps to human chromosome 2q32.2. Defects to this gene result in HIBCH deficiency (HIBCHD), known alternatively as deficiency of _-hydroxyisobutyryl CoA deacylase or methacrylic aciduria. HIBCHD is characterised by the accumulation of methacrylyl-CoA, a highly reactive compound that undergoes addition reactions with free sulfhydryl groups. Phenotypic symptoms include early deterioration of neurological function, delayed motor skill development and hypotonia.
UOM: 1 * 100 µl
Image Unavailable-BOSSBS-15484R-A750

Anti-HIBCH Rabbit Polyclonal Antibody (ALEXA FLUOR® 750)

Catalog Number: (BOSSBS-15484R-A750)
Supplier: Bioss
Description: HIBCH is a 386 amino acid protein belonging to the enoyl-CoA hydratase/isomerase family. Localising to the mitochondria, HIBCH is highly expressed in liver and kidney, with lower levels found in heart, muscle and brain. HIBCH hydrolyses HIBYL-CoA, a saline catabolite, and _-hydroxypropionyl-CoA, an intermediate in the minor pathway involved in the metabolism of proprionate. Existing as two alternatively spliced isoforms, the gene encoding HIBCH maps to human chromosome 2q32.2. Defects to this gene result in HIBCH deficiency (HIBCHD), known alternatively as deficiency of _-hydroxyisobutyryl CoA deacylase or methacrylic aciduria. HIBCHD is characterised by the accumulation of methacrylyl-CoA, a highly reactive compound that undergoes addition reactions with free sulfhydryl groups. Phenotypic symptoms include early deterioration of neurological function, delayed motor skill development and hypotonia.
UOM: 1 * 100 µl
Product Image-WAPR80-2004-53

Accessories for colorimeters, CO 7500 Colourwave and CO 8000 Biowave

Catalog Number: (WAPR80-2004-53)
Supplier: Biochrom
Description: Disposable methacrylate cells, 10 mm pathlength, 2,5 ml
UOM: 1 * 100 items
Product Image-SARS67.746

Plastic cuvettes

Supplier: SARSTEDT
Description: Cuvettes in poly(methyl methacrylate) (PMMA) or polystyrene (PS).

Product Image-R79474

Anion exchange HPLC columns, PRP-X500

Supplier: HAMILTON BONADUZ
Description: PRP-X500 is a superficially porous polymeric anion exchange column designed for the separation, purification and isolation of proteins, peptides and DNA/RNA. The methacrylate polymeric coating of the PRP-X500 provides a more hydrophilic surface, preventing hydrophobic interaction sample losses.

Image Unavailable-8.43784.0500

Benzyl propionate for synthesis, Sigma-Aldrich®

Catalog Number: (8.43784.0500)
Supplier: Merck
Description: Benzyl propionate for synthesis, Sigma-Aldrich®
UOM: 1 * 500 mL
Image Unavailable-8.40106.0025

N-Benzyl-N-methylaniline, Sigma-Aldrich®

Catalog Number: (8.40106.0025)
Supplier: Merck
Description: N-Benzyl-N-methylaniline, Sigma-Aldrich®
UOM: 1 * 25 mL
Image Unavailable-50475-5ML

Benzyl acetate, Supelco®

Catalog Number: (50475-5ML)
Supplier: Merck
Description: Benzyl acetate, Supelco®
UOM: 1 * 5 mL
Image Unavailable-8.16125.0500

Benzyl butyrate for synthesis, Sigma-Aldrich®

Supplier: Merck
Description: Benzyl butyrate for synthesis, Sigma-Aldrich®

Image Unavailable-8.18701.1000

Benzyl benzoate for synthesis, Sigma-Aldrich®

Supplier: Merck
Description: Benzyl benzoate for synthesis, Sigma-Aldrich®

Image Unavailable-8.01809.2500

Benzyl chloride for synthesis, Sigma-Aldrich®

Supplier: Merck
Description: Benzyl chloride for synthesis, Sigma-Aldrich®

Image Unavailable-08421-25ML-F

Benzyl alcohol, Supelco®

Supplier: Merck
Description: Benzyl alcohol, Supelco®

Image Unavailable-8.14688.0250

Benzyl chloroformate stabilised, Sigma-Aldrich®

Catalog Number: (8.14688.0250)
Supplier: Merck
Description: Benzyl chloroformate stabilised, Sigma-Aldrich®
UOM: 1 * 250 mL
Image Unavailable-8.41566.0025

Benzyl phenyl sulphide for synthesis, Sigma-Aldrich®

Catalog Number: (8.41566.0025)
Supplier: Merck
Description: Benzyl phenyl sulphide for synthesis, Sigma-Aldrich®
UOM: 1 * 25 g
Image Unavailable-8.22259.1000

Benzyl alcohol for synthesis, Sigma-Aldrich®

Supplier: Merck
Description: Benzyl alcohol for synthesis, Sigma-Aldrich®

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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.
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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