HIBCH anticorps (AA 251-350) (AbBy Fluor® 594)

N° du produit ABIN2814339

Détail du produit anti-HIBCH anticorps (AbBy Fluor® 594)

Antigène: HIBCH (3-hydroxyisobutyryl-CoA Hydrolase (HIBCH))

Épitope: AA 251-350

Reactivité: Humain

Hôte: Lapin

Clonalité: Polyclonal

Conjugué: Cet anticorp HIBCH est conjugé à/à la AbBy Fluor® 594

Application: Western Blotting (WB), Immunofluorescence (Cultured Cells) (IF (cc)), Immunofluorescence (Paraffin-embedded Sections) (IF (p))

Homologie: Human

Purification: Purified by Protein A.

Immunogène: KLH conjugated synthetic peptide derived from human HIBCH

Isotype: IgG

Information d'application

Indications d'application: IF(IHC-P) 1:50-200
IF(IHC-F) 1:50-200
IF(ICC) 1:50-200

Restrictions: For Research Use only

Stockage

Format: Liquid

Concentration: 1 μg/μL

Buffer: Aqueous buffered solution containing 0.01M TBS ( pH 7.4) with 1 % BSA, 0.03 % Proclin300 and 50 % Glycerol.

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE, which should be handled by trained staff only.

Stock: -20 °C

Stockage commentaire: Store at -20°C. Aliquot into multiple vials to avoid repeated freeze-thaw cycles.

Date de péremption: 12 months

Détails sur HIBCH

Antigène: HIBCH (3-hydroxyisobutyryl-CoA Hydrolase (HIBCH))

Autre désignation: HIBCH (HIBCH Produits)

Synonymes: anticorps DDBDRAFT_0189353, anticorps DDBDRAFT_0233831, anticorps DDB_0189353, anticorps DDB_0233831, anticorps T11J7.4, anticorps T11J7_4, anticorps T11J7.5, anticorps T11J7_5, anticorps HIBYLCOAH, anticorps 2610509I15Rik, anticorps AI648812, anticorps zgc:110824, anticorps 3-hydroxyisobutyryl-coenzyme A hydrolase, anticorps 3-hydroxyisobutyryl-coenzyme a hydrolase,putative, anticorps 3-hydroxyisobutyryl-Coenzyme A hydrolase, anticorps 3-hydroxyisobutyryl-CoA hydrolase (predicted), anticorps 3-hydroxyisobutyryl-CoA hydrolase, anticorps ATP-dependent caseinolytic (Clp) protease/crotonase family protein, anticorps putative 3-hydroxyisobutyryl-coenzyme A hydrolase, anticorps 3-hydroxyisobutyryl-CoA hydrolase L homeolog, anticorps PVX_100950, anticorps CpipJ_CPIJ012030, anticorps PKH_145800, anticorps hibch, anticorps MCYG_05070, anticorps SPBC2D10.09, anticorps CNA02940, anticorps LACBIDRAFT_292320, anticorps SJAG_00944, anticorps VDBG_07520, anticorps AT2G30650, anticorps AT2G30660, anticorps LBRM_32_3920, anticorps HIBCH, anticorps Hibch, anticorps hibch.L

Sujet:

Synonyms: 3 hydroxyisobutyryl Coenzyme A hydrolase, 3 hydroxyisobutyryl Coenzyme A hydrolase, mitochondrial, 3-hydroxyisobutyryl-CoA hydrolase, 3-hydroxyisobutyryl-coenzyme A hydrolase, BETA HYDROXYISOBUTYRYL COENZYME A HYDROLASE, HIB CoA hydrolase, HIB-CoA hydrolase, HIBCH, HIBCH_HUMAN, HIBCoA hydrolase, HIBYL CoA H, HIBYL CoAH, HIBYL-CoA-H, HIBYLCoA H, HIBYLCoAH, mitochondrial, HIBCH_HUMAN.

Background: HIBCH is a 386 amino acid protein belonging to the enoyl-CoA hydratase/isomerase family. Localizing to the mitochondria, HIBCH is highly expressed in liver and kidney, with lower levels found in heart, muscle and brain. HIBCH hydrolyzes 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 characterized 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.

ID gène: 26275

Pathways: Monocarboxylic Acid Catabolic Process