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Image Unavailable-BOSSBS-15484R-HRP

Anti-HIBCH Rabbit Polyclonal Antibody (HRP)

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.
Catalog Number: BOSSBS-15484R-HRP
UOM: 1 * 100 µl
Supplier: Bioss
Image Unavailable-BOSSBS-15484R-CY3

Anti-HIBCH Rabbit Polyclonal Antibody (Cy3)

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.
Catalog Number: BOSSBS-15484R-CY3
UOM: 1 * 100 µl
Supplier: Bioss
Image Unavailable-BOSSBS-15484R-A750

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

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.
Catalog Number: BOSSBS-15484R-A750
UOM: 1 * 100 µl
Supplier: Bioss
Product Image-WAPR80-2004-53

Accessories for colorimeters, CO 7500 Colourwave and CO 8000 Biowave

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

Plastic cuvettes

Description: Cuvettes in poly(methyl methacrylate) (PMMA) or polystyrene (PS).
Catalog Number: SARS67.746
UOM: 1 * 2.000 items
Supplier: SARSTEDT
Product Image-R79474

Anion exchange HPLC columns, PRP-X500

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.
Catalog Number: R79474
UOM: 1 * 1 items
Supplier: HAMILTON BONADUZ
Image Unavailable-8.43784.0500

Benzyl propionate for synthesis, Sigma-Aldrich®

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

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

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

Benzyl acetate, Supelco®

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

Benzyl butyrate for synthesis, Sigma-Aldrich®

Description: Benzyl butyrate for synthesis, Sigma-Aldrich®
Catalog Number: 8.16125.0500
UOM: 1 * 500 mL
Supplier: Merck
Image Unavailable-8.14688.0250

Benzyl chloroformate stabilised, Sigma-Aldrich®

Description: Benzyl chloroformate stabilised, Sigma-Aldrich®
Catalog Number: 8.14688.0250
UOM: 1 * 250 mL
Supplier: Merck
Image Unavailable-8.18701.1000

Benzyl benzoate for synthesis, Sigma-Aldrich®

Description: Benzyl benzoate for synthesis, Sigma-Aldrich®
Catalog Number: 8.18701.1000
UOM: 1 * 1 L
Supplier: Merck
Image Unavailable-8.01809.2500

Benzyl chloride for synthesis, Sigma-Aldrich®

Description: Benzyl chloride for synthesis, Sigma-Aldrich®
Catalog Number: 8.01809.2500
UOM: 1 * 2,5 L
Supplier: Merck
Image Unavailable-08421-25ML-F

Benzyl alcohol, Supelco®

Description: Benzyl alcohol, Supelco®
Catalog Number: 08421-25ML-F
UOM: 1 * 25 mL
Supplier: Merck
Image Unavailable-8.41566.0025

Benzyl phenyl sulphide for synthesis, Sigma-Aldrich®

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

Benzyl alcohol for synthesis, Sigma-Aldrich®

Description: Benzyl alcohol for synthesis, Sigma-Aldrich®
Catalog Number: 8.22259.1000
UOM: 1 * 1 L
Supplier: Merck
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