VWR International - Chemicals and laboratory scientific supplies

Anti-HIBCH Rabbit Polyclonal Antibody (ALEXA FLUOR® 680)

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-A680

UOM: 1 * 100 µl

Supplier: Bioss

Sale

Clearance

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

Sale

Clearance

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

Sale

Clearance

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

Sale